We examined clinical and histopathologic characteristics in 41 patients. Medical records were assessed for presentation, clinical diagnosis, associated urothelial carcinoma, radiation treatment, tobacco use, immunologic/urologic disorder, and treatment strategy/outcome. Results: The mean age was 68 (range 28-87 years). Presenting symptoms were: pain (37 %), hematuria (27 %), and dysuria (20 %), in contrast to asymptomatic (32 %). Clinical diagnosis favored malignancy in 10 % of cases. Concurrent or subsequent urothelial carcinoma was present for five patients (12 %), though none developed urethral carcinoma. Histologic features included mixed hyperplastic urothelial and squamous lining, overlying a variably fibrotic, edematous, inflamed, and vascular stroma. Invaginations of urothelium extending into the stroma were common (68 %), showing rounded nests with cystic or glandular luminal spaces, similar to urethritis cystica/glandularis, without intestinal metaplasia. Two lesions included an organizing thrombus, one with intravascular papillary endothelial hyperplasia. Twenty patients were treated with topical medications without resolution. Three lesions recurred (7 %) after excision. Conclusion: Urethral caruncle is an uncommon lesion that may clinically mimic benign and malignant conditions, making tissue diagnosis critically important.