Small cell carcinomas are well-recognized tumors known to occur predominantly in the lung. These neoplasms occasionally are associated with a variety of symptoms caused by hormones and other products produced by the tumor cells (paraneoplastic syndromes). However, in the gynecologic tract such neoplasms are extremely rare. The authors report the case of an elderly woman who presented with visual disturbances caused by a primary small cell carcinoma of the uterus.