Adult rhabdomyoma is an uncommon benign neoplasm of striated muscle. Although adult rhabdomyoma has a preference for occurring at the head and neck region, occurrence in the tongue base is rare. We presented one such unusual case of 70-year-old male patient who had had lump sensation of throat and progressive dysphagia for 2 years. Flexible endoscopy revealed a huge mass with smooth surface and wide base occupied the tongue base. Computerized tomography showed a homogenous soft tissue mass over the tongue base and protruding to pharyngeal space. Incision biopsy and subsequent transsoral debulking of the tumor revealed adult rhabdomyoma. The patient recovered smoothly with small residual tumor 4 months after surgery. Although rhabdomyoma is a rare soft tissue tumor, it should be one of list in the differential diagnosis of tumors in the head and neck region. Diagnosis of adult rhabdomyoma is based on clinical history and histopathological survey. The differential diagnosis includes granular cell tumor and rhabdomyosarcoma. After complete excision, the recurrence is rare.