We report a case of a chronic expanding hematoma caused by an angiomyolipoma of the spleen in a patient diagnosed with tuberous sclerosis in infancy. Computed tomography showed large bilateral renal angiomyolipomas. A splenic mass that increased in size during the follow-up period of 62 months was also noted. A large subcapsular hematoma of the spleen finally developed, and a splenectomy was performed. The splenic mass consisted of a chronic hematoma with prominent granulation tissue, which was considered to be caused by repeated bleeding from a small angiomyolipoma in the spleen.