Rhabdomyoma in adults is a rare benign neoplasm of the skeletal muscles generally arising in the pharyngolaryngeal region. These slow-growing tumors remain asymptomatic for a long period. We report a case of basilingual rhabdomyoma. Computed tomography and magnetic resonance imaging demonstrated a well-encapsulated tumor with a line of cleavage between the base of the tongue muscles. Deep transmucosal biopsies were obtained. Well-differentiated skeletal muscle cells with large eosinophilic granulular cytoplasm and cross striations gave the histologic diagnosis. Complete endoscopic resection was successful. The differential diagnoses of rhabdomyoma in adults are myoblastoma or Abrikossof tumor, rhabdomyosarcoma and hibernoma. The risk of late recurrence implies long-term clinical and radiologic follow-up.