Abstract
Persistent Müllerian duct syndrome is a rare genetic disorder characterized by a male with retained Müllerian structures. Remnant excision must be considered due to the possibility of malignant degeneration. We review a case of delayed diagnosis in a 25-year-old man presenting with hematuria. Preoperative counseling must emphasize the risk of malignancy versus the risks to fertility. The da Vinci robot offers a novel, safe approach for excision of the relevant Müllerian structures. Dissection should be limited to structures superior to the cavernosal neurovascular bundles to preserve the continence and erectile function. A semen analysis is recommended preoperatively to determine effects on fertility.