Rhabdomyosarcoma is an uncommon tumor which rarly originates in the temporal bone space. Though rare, rhabdomyosarcoma is one of the most common malignant mesenchyml neoplasms of childhood. Horne and Enterline offered a classification of rhabdomyosarcoma which is fairly universally accepted today, and is as follows ; 1) Pleomorphic type 2) Alveolar type 3) Embryonal type 4) Botryoid type. Embryonal or Botryoid types are primarily lesions of children and infants. The most common site of the embryonal rhabdomyosarcoma is the lower urogenital tract, less in the orbit and nasopharynx. Otolaryngologists pay attention to the following points in children ; a) Granulation tissue in the external auditory meatus with no preceding history of infection, biopsy is indicated. b) The presentation of faical nerve paralysis with no clear-cut history of otitis media or mastoiditis consideration as to the possibility of this tumor being the causes. c) X-ray evidence of cloudy mastoid or bone destruction out of proportion to the clinical symptomatology. At the present time rhabdomyosarcoma is treated with radical operation, radiation and cytotoxic agents. Though the best prognosis, radical operation is impractical due to the location of this particular tumor. Inspite of the treatment the prognosis is very poor because of the rapid progression and high incidence of recurrence. A 6-year old male child was admitted to the Dept. of E.N.T., S.N.U.H. through O.P.D. with vomiting, headache, otorrhea in right ear and right side facial palsy on July 23rd, 1970. The right external auditory canal was filled with granulation tissue and lymph nodes were palpable on right side of the neck. The mastoid X-ray films revealed marked destruction of the mastoid region. Radical mastoidectomy was done under the impression of otogenic meningitis and for rule out tumor. Temporal bone was markedly destructed and exposure of the dura and the lateral sinus was noted. The petrous portion was also involed. Biopsy report revealed rhabdomyosarcoma of the embryonal type.