Abstract: Prostate cancer, with an estimated 233,000 new cases and 29,480 deaths in 2014, is the most common malignancy among men in the United States and only second to lung cancer as the cause of cancer-related deaths (CA Cancer J Clin 2014;64:9–29). While conventional (acinar) adenocarcinoma constitutes the vast majority of prostate cancers, rare variants including neuroendocrine tumors have been recognized by the World Health Organization (World Health Organization Classification of Tumours; Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs. Lyon, France: IARC Press; 2004) and the American Joint Commission on Cancer (The AJCC Staging Manual. 7th ed. New York: Springer; 2007). Statistically speaking, when an unusual histologic pattern is encountered, one should always consider the possibility of direct extension from adjacent organs and/or metastasis from distant sites. In this article, a brief presentation of 2 cases is followed by an overall review of the spectrum of neuroendocrine tumors of the prostate.