Small cell lung carcinoma (SCLC), also known as high-grade neuroendocrine tumor of the lung, is exceedingly rare in the pediatric population. SCLC is usually fast growing and often has metastasized at diagnosis. It frequently responds well to therapy initially, however, has a high relapse and mortality rate. There are limited published data on SCLC in children and no existing pediatric treatment protocols. In this report, we present a case of extensive stage SCLC in a 15-year-old boy who responded to single-agent gemcitabine therapy and review similar cases reported in the medical literature.