Primary leiomyosarcomas of the gastrointestinal (GI) tract are extremely rare and highly aggressive neoplasms, and only a small number of true cases have been reported since the concept of GI stromal tumors was established. Here, we report a case of a primary leiomyosarcoma of the transverse colon. A 46-year-old Japanese male with a large mass in the right upper abdomen was admitted to our hospital. Computed tomography and magnetic resonance imaging revealed long segments of wall thickening of the transverse colon with large consecutive tumors measuring 12 cm in diameter. A projecting irregular mass with marked mucosal necrosis was found on colonoscopy. Pathological examination revealed a spindle cell tumor growing circumferentially and transmurally to replace the muscularis propria in the transverse colon. The spindle cells were positive for smooth muscle actin, and negative for KIT, CD34, DOG-1, and S-100 protein. The patient has shown repeat recurrence in spite of sufficient surgical excision being promptly performed.