[Congenital dyserythropoietic anemia]

The author submits the history and classification criteria of congenital dyserythropoietic anaemia and presents at the same time an observation of one of his female patients who was originally dispensarized as hereditary spherocytosis. Failure of splenectomy led to revision of the finding in bone marrow and a change of diagnosis to congenital dyserythropoietic anaemia type II. The patient died at the age of 27 years from hepatic failure as a result of haemosiderosis.