Nineteen patients with primary ethmoid sinus malignancies were treated at the Cleveland (Ohio) Clinic Foundation between 1976 and 1989. Pathologic diagnoses included adenocarcinoma (eight), sarcoma (four), squamous cell carcinoma (three), mucoepidermoid carcinoma (two), adenoid cystic carcinoma (one), and undifferentiated carcinoma (one). All patients underwent surgical resection: 13 had craniofacial resection, four had craniofacial resection/orbital exenteration, one had radical ethmoidectomy/maxillectomy/orbital exenteration, and one had transantral ethmoidectomy. Twelve patients had combined treatment with radiation therapy. Ten patients were alive with no evidence of disease. A trend toward improved prognosis is associated with negative surgical margins. Preservation of the globe was not associated with local recurrence at this site. A poor prognosis was noted with involvement of the dura, brain, nasopharynx, or sphenoid sinus.