[Persistent Mullerian duct syndrome]

We report on a 14-year-old boy who was admitted to the emergency department for acute retention of urine. At age 1 year, he had been submitted to surgery for left cryptorchidism and left-sided herniation, associated with hypospadias of the glans penis. Radiologic and endoscopic work up revealed a large retrovesical cavity with septation in its upper portion communicating with the prostatic urethra. These structures were removed and pathologic examination disclosed remnants of Müllerian ducts. This uncommon disease entity whose etiopathogenesis is ascribed to a deficient activity of the Müllerian inhibiting factor (MIF) classically presents in phenotypically male subjects with unilateral cryptorchidism, contralateral herniation, and persistent Müllerian remnants. The literature is reviewed, highlighting the diagnostic and therapeutic aspects of this syndrome.