The haemophagocytic syndrome is defined as a proliferation of phagocytic macrophages in the bone marrow, lymph nodes and spleen. Clinically, it is characterised by fever and pancytopenia. We present here a case of haemophagocytic syndrome after liver transplantation in a 63-year-old man who had undergone transplantation for autoimmune hepatitis. One month after liver transplantation, he developed ascites, fever and progressive pancytopenia. Bone marrow biopsy showed proliferation of non-neoplastic histiocytes, demonstrating phagocytosis of haemopoietic cells. No infectious or neoplasm-associated disease was found. Several kinds of treatment were attempted, but the course was fatal. The haemophagocytic syndrome is uncommon after liver transplantation, but this diagnosis has to be kept in mind in cases of pancytopenia of unknown origin.