Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood, while RMS from the urinary tract has rarely been reported. Aspects of the manifestation, diagnosis, and treatment of these tumors are discussed in the case of a girl with rapidly progressive RMS from the urethral tract. She was treated with a two-stage surgical procedure and chemotherapy. At the latest follow-up visit at 18 months after treatment, the patient had no evidence of disease on clinical examination or imaging studies. The present case underscores that careful history, physical examination, and laboratory tests should be performed, in additional to using adequate tissue for routine pathologic examination, before making the diagnosis. A combined approach to treating RMS using multidrug chemotherapy and surgery has markedly improved survival.