Between November 1976 and May 1990, twenty-six patients with Budd-Chiari syndrome underwent 28 liver transplants in the Cambridge-Kings College Hospital program. Twelve patients were male, average age 36.6 (range 19-54) and fourteen were female, average age 26 (range 10-47). Possible aetiological factors were identified in 13 cases; polycythaemia rubra vera (4), megakaryocytosis (2), antithrombin III deficiency (1), trauma (1), pregnancy (1), oral contraceptive pill (4). Because of the high incidence of thrombotic complications a protocol of early anticoagulation was introduced early in the series commencing with subcutaneous heparin and progressing to formal full anticoagulation with heparin and then long term anticoagulation with warfarin. Twelve patients have died giving one, three and five year actuarial survival figures of 69.2%, 69.2% and 49.7% respectively, there are currently six patients alive beyond five years. Orthotopic liver grafting in Budd-Chiari syndrome and subsequent anticoagulant thus represents a major treatment option with good long term survival and is the optimal treatment for patients with end stage liver disease.