A 51-year-old man had epidermoid carcinoma in the middle third of the esophagus. The esophagus was resected. On histologic examination there was a granular cell tumor in the lower third of the esophagus. The patient died 4 months after surgery from pulmonary failure. Granular cell tumors are generally benign. There have been 86 cases involving the esophagus discussed in the literature; 7 cases were associated with bronchial [3], esophageal [2], gastric [1] or otolaryngeal [1] carcinoma and 1 with a lymphoma. Our case emphasizes the need to evaluate the entire esophagus when a lesion is identified. The occurrence of granular cell tumor of the esophagus justifies prolonged surveillance in order not to neglect an associated tumor.