Portal vein thrombosis (PVT) is a rare condition with an incidence of 2–4 cases per 100,000 inhabitants. It is commonly associated with chronic liver diseases, such as cirrhosis, and other conditions like hepatobiliary malignancies, infections, or myeloproliferative disorders. PVT can be classified as acute or chronic, with acute PVT involving sudden occlusion of the portal vein and chronic PVT developing from unresolved acute cases. The main differential diagnosis includes malignant portal vein invasion, often by hepatocellular carcinoma, or constriction by pancreatic or cholangiocarcinoma. The management of PVT primarily involves anticoagulation to prevent clot extension and promote recanalization, reducing the risk of complications like portal hypertension and intestinal infarction. Anticoagulant therapy is generally recommended for at least three to six months, and may be continued indefinitely in cases of permanent pro-coagulant conditions or thrombosis extending to mesenteric veins. Risk factors for PVT include chronic liver disease, certain cancers, and thrombophilic disorders. PVT is associated with various conditions, including infections, sepsis, and inflammatory bowel disease. It is also linked to liver diseases such as non-alcoholic fatty liver disease and metabolic dysfunction-associated steatotic liver disease. PVT is more common in patients with cirrhosis and is related to the severity of liver disease. Diagnosis of PVT involves imaging techniques such as ultrasound, CT, and MRI, with ultrasound being the first-line method. Laboratory tests are limited in diagnosing acute PVT, but may show elevated inflammatory markers or signs of liver failure. Differential diagnosis includes malignant portal vein invasion and other conditions that mimic PVT. Anticoagulant therapy is the main treatment for PVT, with direct oral anticoagulants (DOACs) being preferred over warfarin due to their safety profile and ease of use. DOACs have shown effectiveness in treating PVT, with higher rates of recanalization and lower bleeding risks compared to other anticoagulants. In cirrhotic patients, anticoagulation must be carefully managed due to the increased risk of bleeding. The duration of anticoagulation is typically three to six months, but may be extended based on individual patient factors. Alternative treatments, such as thrombolysis or surgical thrombectomy, are considered in specific cases but are associated with risks. Complications of PVT include variceal bleeding, hepatic encephalopathy, ascites, and cholangitis, which require appropriate management. In summary, PVT is a complex condition requiring careful evaluation and management, with anticoagulation being the primary treatment. The management of PVT is influenced by the underlying condition, the severity of the disease, and the patient's overall health status.Portal vein thrombosis (PVT) is a rare condition with an incidence of 2–4 cases per 100,000 inhabitants. It is commonly associated with chronic liver diseases, such as cirrhosis, and other conditions like hepatobiliary malignancies, infections, or myeloproliferative disorders. PVT can be classified as acute or chronic, with acute PVT involving sudden occlusion of the portal vein and chronic PVT developing from unresolved acute cases. The main differential diagnosis includes malignant portal vein invasion, often by hepatocellular carcinoma, or constriction by pancreatic or cholangiocarcinoma. The management of PVT primarily involves anticoagulation to prevent clot extension and promote recanalization, reducing the risk of complications like portal hypertension and intestinal infarction. Anticoagulant therapy is generally recommended for at least three to six months, and may be continued indefinitely in cases of permanent pro-coagulant conditions or thrombosis extending to mesenteric veins. Risk factors for PVT include chronic liver disease, certain cancers, and thrombophilic disorders. PVT is associated with various conditions, including infections, sepsis, and inflammatory bowel disease. It is also linked to liver diseases such as non-alcoholic fatty liver disease and metabolic dysfunction-associated steatotic liver disease. PVT is more common in patients with cirrhosis and is related to the severity of liver disease. Diagnosis of PVT involves imaging techniques such as ultrasound, CT, and MRI, with ultrasound being the first-line method. Laboratory tests are limited in diagnosing acute PVT, but may show elevated inflammatory markers or signs of liver failure. Differential diagnosis includes malignant portal vein invasion and other conditions that mimic PVT. Anticoagulant therapy is the main treatment for PVT, with direct oral anticoagulants (DOACs) being preferred over warfarin due to their safety profile and ease of use. DOACs have shown effectiveness in treating PVT, with higher rates of recanalization and lower bleeding risks compared to other anticoagulants. In cirrhotic patients, anticoagulation must be carefully managed due to the increased risk of bleeding. The duration of anticoagulation is typically three to six months, but may be extended based on individual patient factors. Alternative treatments, such as thrombolysis or surgical thrombectomy, are considered in specific cases but are associated with risks. Complications of PVT include variceal bleeding, hepatic encephalopathy, ascites, and cholangitis, which require appropriate management. In summary, PVT is a complex condition requiring careful evaluation and management, with anticoagulation being the primary treatment. The management of PVT is influenced by the underlying condition, the severity of the disease, and the patient's overall health status.