Extrapulmonary small cell carcinoma of the stomach is a rare and aggressive malignancy with a poor prognosis that was first described in 1976 by Matsusaka et al. In 1989 it was recognized by the World Health Organization as an independent entity affecting the stomach. Pure and composite are the two types of gastric small cell carcinoma reported in the literature. We report a case of a 68-year-old African American male with metastatic pure-type extrapulmonary small cell cancer of the stomach. The primary lesion measured approximately 7 cm in diameter on endoscopy. The diagnosis was made on the basis of characteristic histological features of small, round, oval lymphocyte-like cells with hyperchromatic nuclei, and scant cytoplasm consistent with small cell carcinoma. Gastric small cell carcinoma is typically diagnosed at an advanced stage as demonstrated in our case. We conducted a literature review discussing the two types of extrapulmonary small cell carcinoma of the stomach and their prevalence.