The antiphospholipid syndrome involves long-term persistence of serum antiphospholipid antibodies and hypercoagulability manifested by venous or arterial thrombosis, recurrent pregnancy loss or thrombocytopenia. Two forms have been described: the “primary syndrome” where there is no evidence of an underlying disease, and the “secondary syndrome”, mainly in the setting of systemic lupus eryhematosus. Classic features of antiphospholipid syndrome include cutaneous signs, such as livedo reticularis, splinter hemorrhages, superficial thrombophlebitis, and leg ulcers; venous thrombotic events, such as cerebral or retinal vein thrombosis; arterial thrombotic events, such as transient attacks or ischemic infarcts, recurrent spontaneous early-term abortions, and thrombocytopenia. Also various hepatic manifestations have been reported with antiphospholipid syndrome in literature, including Budd-Chiari syndrome, hepatic-veno-occlusive disease and occlusion of small hepatic veins, nodular regenerative hyperplasia and portal hypertension. Herein we present a case of non-cirrhotic portal hypertension associated with primary antiphospholipid syndrome which this coexisting is rarely seen in the literature.