Primary small cell carcinoma of the prostate is rare. A case of primary small cell prostate cancer treated with radiation and chemotherapy is presented, and 33 previously published case reports are reviewed. Most of the patients (61%) had mixed tumors (small cell and adenocarcinoma) at diagnosis or had a history of adenocarcinoma of the prostate. Prostate specific antigen (PSA) data was available in 11 patients and was abnormal in 4 (36%). Once small cell carcinoma was diagnosed, 70% of patients had metastatic disease. Visceral metastases were common. Only one of seven patients responded to hormonal therapy, and two of eight patients responded to chemotherapy. Overall prognosis was poor.