Small cell cancer(SCC) is a distinct clinicopathological entity. Although it has commonly pulmonary origin, SCC may also originate in extrapulmonary organs, including gastrointestinal tract, head and neck region, urogenital system, and occasionally unknown primary origin, which is known as extrapulmonary small cell cancer(EPSCC). Given the rarity of the disease, no large-sized randomized trial has been performed to guide the treatment of patients with EPSCC. This lack of data and its pathological similarity to the small cell lung cancer(SCLC) led most investigators to adopt similar principles of SCLC to the treatment of EPSCC. However, difference of clinical course and frequency of distant metastases warrant differential therapeutic approaches. In this paper, we reviewed the available data concerning the treatment of EPSCC originated at different sites.