An 18-month-old girl was referred for evaluation of vaginal bleeding and a renal mass. Ultrasonography and computed tomography revealed multiple simple cysts within the left kidney and a normal right kidney. At vaginoscopy, a 1-cm frond-like papillary lesion overlying the cervix was identified. The pathologic diagnosis was mullerian papilloma, a rare benign tumor of the vagina and uterine cervix. None of the approximately 40 previously reported cases have been associated with renal anomalies, despite the historical classification of these lesions as mesonephric papillomas. We report the first patient with both a renal anomaly and mullerian papilloma of the uterine cervix.