Portal vein thrombosis (PVT) is a rare disorder that is associated with a variety of underlying conditions, of which liver cirrhosis, malignancy and myeloproliferative disorders are the most common. Based on clinical presentation and results of imaging, two different entities can be identified, acute and chronic PVT. Anticoagulation therapy is recommended for all patients with acute PVT in an attempt to prevent further thrombosis and to promote recanalisation of the obstructed veins. Chronic PVT is characterised by the presence of a portal cavernoma and development of portal hypertension. Bleeding from ruptured oesophageal or gastric varices is the main complication of portal hypertension in these patients. Both endoscopic therapy and beta-adrenergic blockade are used for the prevention and treatment of gastrointestinal bleeding. In the absence of bleeding, continuous anticoagulant therapy should be considered for the group of chronic PVT patients in whom an underlying prothrombotic factor can be identified. With adequate management of complications and concurrent diseases, prognosis of PVT is good in patients without underlying cirrhosis or malignancies.