Small cell prostate neuroendocrine carcinoma (SCPC) is a rare and highly aggressive malignant tumor. We present a case of a 52-year-old Iranian man, presenting with complaints of occasional gross hematuria and perineal pain for 6 months. PSA was 0.8 ng/ml. A digital rectal examination found a huge and hard prostate mass. He underwent a transrectal ultrasound-guided (TRUS) biopsy of the prostate. Histopathology showed high-grade small cell neuroendocrine carcinoma. Immunohistochemical markers were positive for synaptophysin with a Ki67 index of almost 100%. However, CD56 and chromogranin A markers were negative. Magnetic resonance imaging (MRI) of the prostate showed a prostate mass with invasion to the rectum, while contrast-enhanced computed tomography of the thorax, abdomen, and pelvis (CT TAP) ruled out metastasis. A multidisciplinary team discussion was carried out, and a decision was made for concurrent chemotherapy and radiation (cisplatin and etoposide for 4 cycles and 70 Gy, 35 fractions). There is a lack of consensus on the management of SCPC. The main modality of management in advanced (stage IV) disease is chemotherapy. It is a highly aggressive tumor with a poor prognosis and is not responsive to hormonal therapy.