A 39-year-old Japanese man with a splenic hamartoma who underwent surgery, is reported herein. The patient had a volvulus of the transverse colon and acute Type A hepatitis. His symptoms included fever, abdominal pain and icterus and the serum chemistry showed liver dysfunction and an elevation of hepatitis A antibody levels. Barium enema revealed a volvulus of the transverse colon. Ultrasonogram showed a hypoechoic mass and angiogram showed evidence of stretching of the intrasplenic vessels in the arterial phase and a hypovascular mass lesion in the venous phase. Macroscopically, the tumor was a well-defined, whitish-yellow, hard nodule. Microscopically, the tumor was composed of fibrous scar tissue containing scattered red pulp tissues and the histologic diagnosis was a splenic hamartoma of the fibrous type. The patient's postoperative recovery was uneventful. The clinical features of splenic hamartoma are reviewed in this report and the differential diagnosis is also discussed.