This article reviews the recent literature on extrapulmonary small cell carcinomas. Until now, only four cases have been published in the English literature, two of those in the Southern Medical Journal. Sharing the information on diagnosis and treatment of these cases is important for better understanding and treatment of these patients. The differential diagnosis of pleural masses and the relation to asbestos exposure is briefly discussed. In addition, the available literature on other extrapulmonary localizations of small cell carcinoma is reviewed. The focus is on the available larger series of patients. Small cell carcinoma of the skin is included. This form of small cell carcinoma is often excluded from the larger review series, but can be an important alternative in the diagnostic process of patient evaluation.