Portal vein thrombosis (PVT) is a rare but significant complication of various pathological conditions, including chronic liver diseases, hepatobiliary malignancies, and inflammatory diseases. The incidence of PVT is estimated at 2 to 4 cases per 100,000 inhabitants, with higher prevalence in patients with cirrhosis. PVT can be classified based on anatomical site, degree of venous occlusion, and timing and type of presentation. Management of PVT involves anticoagulation and addressing underlying predisposing conditions. Anticoagulation aims to prevent clot extension and enable recanalization to avoid complications like intestinal infarction and portal hypertension. The treatment duration is generally recommended for at least three to six months, with longer durations advised for patients with underlying pro-coagulant conditions or thrombosis extending to the mesenteric veins. Direct oral anticoagulants (DOACs) are preferred over traditional anticoagulants due to their safety profile and better patient compliance. In cirrhotic patients, anticoagulation therapy must be carefully considered due to the increased risk of bleeding and portal hypertension. Thrombolytic therapy and surgical thrombectomy are alternative treatments but are less commonly used due to their associated risks. Complications of PVT, such as variceal bleeding, hepatic encephalopathy, ascites, and cholangitis, are managed similarly to those without PVT. TIPS procedures may be considered for patients with symptoms associated with portal hypertension that do not respond to other therapies.Portal vein thrombosis (PVT) is a rare but significant complication of various pathological conditions, including chronic liver diseases, hepatobiliary malignancies, and inflammatory diseases. The incidence of PVT is estimated at 2 to 4 cases per 100,000 inhabitants, with higher prevalence in patients with cirrhosis. PVT can be classified based on anatomical site, degree of venous occlusion, and timing and type of presentation. Management of PVT involves anticoagulation and addressing underlying predisposing conditions. Anticoagulation aims to prevent clot extension and enable recanalization to avoid complications like intestinal infarction and portal hypertension. The treatment duration is generally recommended for at least three to six months, with longer durations advised for patients with underlying pro-coagulant conditions or thrombosis extending to the mesenteric veins. Direct oral anticoagulants (DOACs) are preferred over traditional anticoagulants due to their safety profile and better patient compliance. In cirrhotic patients, anticoagulation therapy must be carefully considered due to the increased risk of bleeding and portal hypertension. Thrombolytic therapy and surgical thrombectomy are alternative treatments but are less commonly used due to their associated risks. Complications of PVT, such as variceal bleeding, hepatic encephalopathy, ascites, and cholangitis, are managed similarly to those without PVT. TIPS procedures may be considered for patients with symptoms associated with portal hypertension that do not respond to other therapies.