Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition with high mortality rate besides aggressive multimodal treatment. Underlying triggers of “thrombotic and cytokine storm” include pregnancy, inflammation, trauma, surgery, and infection. The authors present a case of a young female patient with primary antiphospholipid syndrome (APS) who was admitted to the hospital due to abdominal pain caused by ovarian tumor with elevated tumor markers. After the prophylactic anticoagulants and antibiotic treatment, surgery was performed. Suddenly after treatment, her clinical status deteriorated and she died regardless of intensive immunosupresive and anticoagulant therapy attempts. This condition requires all clinical awareness, timely diagnosis, and therapeutical approach, including a better understanding of the pathophysiology that leads to CAPS.