The significant risks associated with total splenectomy have led to interest in the use of partial splenectomy as an alternative surgical therapy for children who have congenital hemolytic anemia. Partial splenectomy is designed to remove enough spleen to gain desired hematologic outcomes while preserving splenic immune function. Although preliminary data demonstrate successful laboratory and clinical outcomes after partial splenectomy in various congenital hemolytic anemias, conclusive data comparing the efficacy of partial splenectomy to total splenectomy are not reported. Based on preliminary data, a definitive clinical trial of partial splenectomy in children who have severe congenital hemolytic anemia may be warranted.