Portal vein thrombosis is a rare occurrence, and often an underlying hyperc
oagulable state can be found. Recently, there has been growing interest and
recognition of the antiphospholipid syndrome in association with acquired
hypercoagulable state. This syndrome consists of the association of lupus a
nticoagulant or antiphospholipid antibodies with arterial or venous thrombo
sis, thrombocytopenia, and spontaneous abortion. We report a case of portal
vein thrombosis associated with the antiphospholipid syndrome. In our pati
ent, chronic liver disease, hepatobiliary infection, abdominal malignancies
, myeloproliferative disorders, and inherited coagulation disorders were ex
cluded. This case report suggests that serum antiphospholipid antibodies sh
ould be investigated in patients with portal vein thrombosis of unexplained
etiology.