Thirty-one patients with malignant tumors of the middle ear and external auditory canal (EAC) were observed at the University of Virginia Hospital from 1956 through 1980. Of 27 patients with carcinoma, 21 had squamous cell carcinoma, 4 had basal cell carcinoma and 2 had adenoid cystic carcinoma. One Ewing's sarcoma and 3 rhabdomyosarcomas occurred in an age group of one to 10 years. The 27 patients with carcinoma are reviewed with regard to clinical presentation, treatment modality, results and complications. The majority (67%) of patients had a history of chronic ear drainage, 22% had a previous mastoidectomy or polypectomy and 7% had an associated cholesteatoma. The treatment modalities employed depended on the extent of disease and the patient's general condition. Eighty percent of patients with carcinoma limited to EAC were alive and well at 5 years, compared to 43% of patients with involvement of the middle ear. Fifty-six percent of patients without invasion of the petrous bone were alive at 5 years compared to only 20% of patients with petrous bone involvement. The data strongly suggest that survival depends on the extent of disease. The corrected disease free 5 year survival rates were 14% for patients who had surgery alone and 50% for those who had surgery and radiotherapy. Of the three patients with advanced disease who received radiotherapy alone, none survived five years. Surgery or radiotherapy alone is not sufficient for most of the cases of carcinoma of the middle ear, since these patients often present with advanced disease. We recommend combined therapy, especially postoperative radiotherapy, except for early lesions that do not involve the mastoid or petrous bone. Other series suggest radiotherapy would be adequate and more effective than surgery for early lesions. Surgery will provide the diagnosis, determine the extent of disease, offer adequate drainage of infected material and relieve most pain before radiation therapy.