Rhabdomyosarcomas are the most common soft tissue tumors in children. Head and neck is one of the most frequently affected site. Within the nonparameningeal, nonorbital head and neck region, rhabdomyosarcomas of the tongue are very rare. We present a 2-year-old boy diagnosed with rhabdomyosarcoma of the tongue. The child was treated with complete surgical resection and chemotherapy. He is followed up with no evidence of disease 6 months after termination of therapy. The literature on rhabdomyosarcoma of the tongue is reviewed.