Primary noncarcinomatous malignant neoplasms of the esophagus are uncommon and data concerning treatment and results are sparse. To evaluate the results of therapy in this group, we reviewed the records of 32 patients with primary esophageal malignant tumors of unusual histologic type. Thirteen patients (41%) had sarcoma, eight (25%) melanoma, and 11 (34%) had oat cell carcinoma. Dysphagia was present in 78% (25/32) of the patients for a median of 13 weeks before diagnosis. Location of the esophageal primary tumor was upper third in four patients (12%), middle third in 12 (38%), and lower third in 16 (50%). Treatment consisted of esophagectomy in 10 of 13 patients with sarcoma (77%), seven of eight with melanoma (88%), and three of 11 with oat cell carcinoma (27%). Patients not undergoing resection received chemotherapy or radiation therapy, or both. The 3- and 5-year survival rates were 46% and 23% for sarcoma (median 20 months), 13% and 0% for melanoma (median 5 months), and 0% and 0% for oat cell carcinoma (median 5 months), respectively. Distant disease was the initial form of recurrence in 73% (11/15) of patients undergoing curative therapy. Surgical resection appears indicated for localized primary esophageal sarcoma. Optimum treatment of primary esophageal melanoma is less clear, but surgical resection may be of benefit in selected patients. Esophageal oat cell carcinoma is a systemic disease necessitating systemic therapy with local therapy reserved for palliation of dysphagia.