Rhabdomyosarcoma of the middle ear is a particularly lethal tumor of childhood. To date, survival data indicate a fatal outcome usually within one year of diagnosis. Adjunct chemotherapy in combination with surgery and radiation offers improved survival in other childhood rhabdomyosarcomas. In this case of middle ear rhabdomyosarcoma, cyclophosphamide, vincristine, and dactinomycin combined chemotherapy were used in conjunction with local surgical extirpation and radiation. The patient has survived 31 months since the diagnosis and remains free of any evidence for tumor regrowth.