We report a 10-month-old girl who, at 4 months, developed a small, reddish, plaquelike lesion on her tongue. This lesion began to enlarge rapidly, resulting in difficulty in swallowing and breathing. On physical examination, there was a large, red, friable, hard in consistency, irregular tumor over the distal portion of her tongue. The histopathology and immunohistochemical findings were consistent with an embryonal rhabdomyosarcoma. The patient was assigned clinical stage I, group III, and began treatment according to the Intergroup Rhabdomyosarcoma Study guidelines, on triple-agent chemotherapy consisting of vincristine, actinomycin D, and cyclophosphamide. She only received four cycles because her parents then refused this treatment. After the tumor size was reduced by chemotherapy, a partial anterior glossectomy was performed. After 30 months of follow-up she has had no recurrences.