Antiphospholipid syndrome (APS), a leading entity in acquired thrombophilia, is characterized by recurrent thrombosis, morbidity in pregnancy and presence of antiphospholipid antibodies (APA). Although the etiopathogenesis is unclear, APA against negatively charged phospholipids and phospholipid-protein complexes are held responsible for the clinical picture. In case of acute thrombosis due to APS, thrombolytic therapy is not a commonly administered treatment option. Here, we present a case with acute thrombosis in the left renal artery showing partial response to thrombolytic therapy.