INTRODUCTION/OBJECTIVES: Our objective was to review our experience with lower urinary tract leiomyomata to determine if presentation, exam and imaging allows for stratification of the treatment of this rare lesion. MATERIALS AND METHODS: A retrospective review of current practice was performed from the Mayo Clinic Rochester database identifying 13 female patients and 10 males from 1995-2008, focusing on the clinical presentation, cystoscopic findings, imaging and operative approaches. RESULTS: Symptomatic patients had larger lesions, were more likely to be females, or had midline periurethral or bladder neck lesions. Cystoscopic findings were uniformly described as submucosal polypoid lesions without surrounding erythema or mucosal involvement, a hallmark in the differential. There were no cases of rapid growth of these lesions. There were no recurrences after complete excision with follow up ranging in some cases for up to 4 years. CONCLUSIONS: Lower urinary tract urethral leiomyomata are benign lesions that can be clearly differentiated on the basis of modern imaging from malignant or other benign lesions. While these lesions can cause obstructive voiding symptoms, resection or excision is required only for relief of symptoms, and not for tissue confirmation. In comparison to the presentation of leiomyosarcoma, this benign mass presents with slowly progressive symptoms, is firm but with rounded contours on exam, mucosal ulceration is absent. Imaging confirms benign appearing, albeit, ectopic muscle tissue. Rare forms of metastatic uterine smooth muscle tumors are also easily differentiated from this lesion. Adoption of a conservative approach for the asymptomatic and benign appearing lesion appears prudent.