Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 4 2 2019 08 06 40 43 Behshad Pazooki Department of Internal Medicine, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran. Hanieh Radkhah Department of Internal Medicine, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran. Alborz Sherafat Department of Cardiology, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran. 2018 12 25 2019 03 12 Portal Vein Thrombosis (PVT), commonly associated with cirrhosis of liver and thrombophilia, is one of the causes of severe abdominal pain. In the absence of non-cirrhotic non-malignant extrahepatic portal vein thrombosis, Myeloproliferative Disease (MPD) and an underlying thrombotic disorder should always be suspected and investigated. Hyperhomocysteinemia has been well-documented to increase the risk of arterial thrombotic events, peripheral arterial disease, and stroke. It is also a risk factor for deep-vein thrombosis. In the general population, association with portal vein thrombosis is very unusual, and only a few cases have been reported. We describe a case of Polycythemia Vera (PV) and hyperhomocysteinemia presenting with severe abdominal pain due to portal vein thrombosis. The patient underwent phlebotomy and was prescribed life-long anticoagulant, aspirin, vitamin B6, vitamin B12, and folic acid, then referred to a hematologist. https://crcp.tums.ac.ir/index.php/crcp/article/view/244 https://crcp.tums.ac.ir/index.php/crcp/article/download/244/312