Abstract. Spontaneous splenic rupture, also referred to as atraumatic splenic rupture, is a rare but life-threatening emergency condition. Without timely diagnosis and treatment, the mortality rate of splenic rupture approaches 100%. The etiology of atraumatic splenic rupture varies; it is reportedly associated with neoplasms or splenic infection, but is rarely encountered in patients with both conditions. We herein report the case of a 58-year-old male patient with acute myeloid leukemia (AML) complicated by splenic tuberculosis (TB), who presented with spontaneous rupture of the spleen. Pathological examination of the resected spleen revealed multifocal granulomatosis with caseous necrosis. However, with timely diagnosis and surgical intervention, the patient recovered successfully and is currently on consolidation therapy. To the best of our knowledge, this is the first case of spontaneous splenic rupture in AML with splenic TB. The relevant literature on spontaneous splenic rupture was also reviewed and the potential etiology and treatment were discussed.