Vaginal Urethra, Clitoral Hypertrophy and Accessory Phallic Urethra: A Rare Syndrome of Female Pseudohermaphroditism

The rare syndrome of idiopathic female pseudohermaphroditism with vaginal urethra, clitoral hypertrophy and accessory phallic urethra is commonly associated with urinary tract obstruction. We report a new case and discuss the characteristic features. The embryogenesis of this condition seems to involve complex interactions between the müllerian duct and urogenital sinus rather than hormonal causes. Treatment is aimed at correcting the masculinized appearance of the genitalia and relieving the frequently present urinary obstruction.