Dyserythropoietic anemias are extremely rare disorders which often are misdiagnosed as megaloblastic anemia, DiGuglielmo's syndrome, refractory sideroblastic anemia, paroxysmal nocturnal hemoglobinuria, or other hemolytic anemias. Dyserythropoietic anemia is now subclassified into four types, designated Types I through IV, depending upon light and ultrastructural morphology, erythrocyte immunology, and the course of the disease. This report details the findings in a case of Type I dyserythropoietic anemia that represented a puzzling case of anemia for over 30 years. Many modes of drug therapy, as well as splenectomy, were not helpful. Interestingly, the family history was non-contributory. A brief review of the literature dealing with dyserythropoietic anemia is included.