AbstractWe present a review of the incidence, differential diagnoses, mechanism of rupture, laboratory findings, and signs and symptoms of splenic rupture in leukemia. The types of leukemia most often associated with ruptured spleen are described. The treatment of splenic rupture in leukemia is splenectomy, with careful evaluation of the need for transfusion with appropriate blood products. The issue of whether such cases should be referred to as “pathologic” or “spontaneous” is discussed. Indications for prophylactic splenectomy in leukemia are also addressed.