A 55-year-old woman presented with massive refractory ascites in the course of idiopathic myelofibrosis. The ascites was exudative, and a cytological examination revealed granulocytes of varying maturity, erythroblasts, and megakaryocytes with trisomy 8. The ascites was assumed to have developed from peritoneal extramedullary hematopoiesis. An abnormal karyotype in the cells in the ascitic fluid, which was the same abnormality as in peripheral blood, helped to prove extramedulary hematopoiesis in this case, which can be an aid in making a differential diagnosis in cases of ascites associated with myelofibrosis. (Internal Medicine 42: 525-528, 2003)