Spindle cell (or pseudosarcomatous) squamous carcinoma (PSC) is a rare malignant neoplasm of the esophagus, potentially capable of causing lymph node and distant metastases. Indications for surgery are the same as for squamous cell carcinoma (SCC) of the esophagus. The aims of this paper were to report a case of endoscopically treated PSC and to review our experience of surgically-treated patients with PSC in order to identify patients potentially suitable for endoscopic treatment. In our series of 4,460 patients with carcinoma of the esophagus observed between 1980 and 2003, 28 (0.6%) had the histological features of PSC. One had a PSC histologically confirmed (8cm-long polyp with a 3cm-large base) and endoscopically treated for high surgical risk. The patient had a close follow-up with endoscopic biopsies and ultrasonography with no local recurrence at 3 years. The overall survival rate was 22% for PSC and 17% for SCC (P = n.s.); after 5 years, the survival rates were 22% and 13%, respectively (P = n.s.). In our opinion the limited tendency to parietal infiltration and the good chance of disclosure in an early stage with endoscopic ultrasonography, justify non-surgical solutions in patients with a high surgical risk, possibly associated with adjuvant chemo- and radiotherapy since lymph node involvement is reported in 50% of cases. The limited number of patients with PSC involved in the present series prevent any significant statistical comparisons between the different groups, but the survival rates were roughly the same in the nonsurgical curative therapy as in the curative resection group, while the chances of survival were significantly lower in patients given palliative surgery and or non-curative treatments (P < 0.05).