Abstract Context.—Carcinoid tumors are exceedingly rare in the genitourinary tract and may occur in the kidney, urinary bladder, urethra, or prostate. Objective.—To review the clinical and pathologic features of carcinoid tumors occurring in the urinary tract and prostate. Data Sources.—We searched the English language literature using MEDLINE and Ovid. Conclusions.—Carcinoid tumors of the urinary tract and prostate share similar morphologic features with their counterparts in other organs. The differential diagnosis includes metastatic carcinoid tumor, paraganglioma, and nested variants of urothelial and prostatic carcinomas. Correlation of the clinical presentation and histopathologic features (including the immunohistochemical profile) will ensure accurate diagnosis of these rare tumors.