Catastrophic Antiphospholipid Syndrome (CAPS) is a rare and life-threatening condition characterized by multiple microthrombosis due to an increased inflammatory response, known as a "thrombotic storm." It typically develops after infection, trauma, or surgery and presents with symptoms such as fever, thrombocytopenia, muscle weakness, visual and cognitive disturbances, abdominal pain, renal failure, and disseminated intravascular coagulation. The presence of antiphospholipid antibodies (aPLs) in the blood is a diagnostic criterion, but their levels can fluctuate, complicating diagnosis. Treatment often involves triple therapy, including anticoagulants, plasmapheresis, and high doses of glucocorticoids, with LMWH being the drug of choice due to its anti-inflammatory and anticoagulant properties. CAPS is a multifactorial disease requiring interdisciplinary care, timely diagnosis, and appropriate prevention. Improved clinical protocols and education for medical personnel can enhance therapeutic approaches and reduce mortality rates. The condition is associated with high mortality, especially in patients with systemic lupus erythematosus (SLE) and those over 36 years old. Preventive measures include anticoagulant prophylaxis before and after surgical interventions, early genetic testing for thrombophilia, and timely delivery in high-risk pregnant women. The relationship between severe coronavirus infections and CAPS is still under investigation, but there are isolated cases suggesting a potential link. Further research is needed to better understand the pathological mechanisms and develop more effective treatments.Catastrophic Antiphospholipid Syndrome (CAPS) is a rare and life-threatening condition characterized by multiple microthrombosis due to an increased inflammatory response, known as a "thrombotic storm." It typically develops after infection, trauma, or surgery and presents with symptoms such as fever, thrombocytopenia, muscle weakness, visual and cognitive disturbances, abdominal pain, renal failure, and disseminated intravascular coagulation. The presence of antiphospholipid antibodies (aPLs) in the blood is a diagnostic criterion, but their levels can fluctuate, complicating diagnosis. Treatment often involves triple therapy, including anticoagulants, plasmapheresis, and high doses of glucocorticoids, with LMWH being the drug of choice due to its anti-inflammatory and anticoagulant properties. CAPS is a multifactorial disease requiring interdisciplinary care, timely diagnosis, and appropriate prevention. Improved clinical protocols and education for medical personnel can enhance therapeutic approaches and reduce mortality rates. The condition is associated with high mortality, especially in patients with systemic lupus erythematosus (SLE) and those over 36 years old. Preventive measures include anticoagulant prophylaxis before and after surgical interventions, early genetic testing for thrombophilia, and timely delivery in high-risk pregnant women. The relationship between severe coronavirus infections and CAPS is still under investigation, but there are isolated cases suggesting a potential link. Further research is needed to better understand the pathological mechanisms and develop more effective treatments.