OBJECTIVE: To review the diagnosis and management of nephrogenic adenoma (NA), an uncommon benign lesion found in the urinary tract. This lesion arises from a proliferation of implanted renal tubular cells. Although more common in adults, it can occur in all ages. NAs can recur and cause significant morbidity in patients. NAs are also a potential diagnostic pitfall as they can clinically and histologically mimic malignancy in the urinary tract. MATERIALS AND METHODS: We performed an Institutional Board Review approved search of our surgical pathology database from 2005 to 2015 for cases of NA. A retrospective chart review was performed with a focus on the clinical, pathologic, and radiographic findings in these patients. RESULTS: We identified 32 cases of NA in 31 patients. Lesions were most common in Caucasian males (male-to-female ratio of 2:1) with an average age at diagnosis of 55 years (range 25-77). Bladder was the most common site of occurrence (81.2%), followed by ureter (9.4%), urethra (6.3%), and intrarenal collecting system (3.1%). Most patients (72%) were symptomatic and presented with hematuria (41%), lower urinary tract symptoms (28%), pelvic or flank pain (6%), hydronephrosis (19%), or urinary incontinence (13%). NA was asymptomatic and identified incidentally in 9 (28%) patients. One patient (3%) had a renal transplant and 8 (26%) patients had diabetes mellitus. Twenty-six (84%) patients were managed with endoscopic resection of their tumors. CONCLUSION: NAs are benign lesions that may cause significant morbidity and mimic malignant tumors. There should be increased suspicion in patients with predisposing factors.