A extrapulmonary small cell carcinoma is a very rare disease, and a primary pleural manifestation is extremely rare. A diagnosis of a small cell carcinoma should be based on the cell morphology, histological pattern, and an immunohistochemical study. We recently experienced a case of small cell carcinoma (SCC) of the pleura in a 59-year-old man who had suffered from right pleuritic chest pain. A histopathological confirmation of SCC was made by a video-associated thoracoscopic lung biopsy. Systemic chemotherapy with etoposide and cisplatin was initiated.