Epithelioid haemangioendothelioma of the liver is an extremely rare tumour which has only recently been recognised. This report describes the clinical, radiological and histological findings in a case of a primary epithelioid haemangioendothelioma of the liver. The case highlights some of the difficulties in establishing the diagnosis, and in its differentiation from Budd-Chiari syndrome and veno-occlusive disease. The patient has subsequently undergone successful orthotopic hepatic transplantation.