SUMMARY. Granular cell tumors (GCTs) are relatively uncommon, usually benign and solitary neoplasms. Until now, about 200 cases of esophageal GCTs have been reported in the literature. We present a rare case of synchronous occurrence of esophageal GCT and moderately differentiated squamous cell carcinoma in a 40-year-old white woman. The GCT was detected incidentally during esophagoscopy undertaken for evaluation of a 4-month history of progressive solid food dysphagia. The gross and microscopic appearance of the GCT was typical. It was localized in the mucosa of the middle esophagus dystally and separately to the cancer. It revealed strong positive immunostaining for vimentin, S-100 protein and neuron-specific enolase, as well as weakly positive focal staining for Ki67 and p53 protein. Although, the coexistence of esophageal GCTs and cancers seems to be coincidental, the necessity of a careful clinical evaluation and a close follow-up of patients with GCT is suggested.