During the last 8 years 54 children with a persistent cloaca have been treated by one of us (A. P.). The most common associated anomalies involved the urinary tract, with renal agenesis, renal dysplasia, vesicoureteral reflux and megaureter encountered most frequently. Four patients demonstrated the rare entity of an opening at the tip of a pseudophallus and 2 also had an accessory urethra in association with the cloacal malformation. These children exhibited some form of masculinization of the external genitalia with the accessory urethra or cloacal channel assuming a phallic position in an enlarged clitoris. No adrenal, metabolic or chromosomal abnormalities were detected and virilization was limited to the genitalia. These cases illustrate the greater predilection of these patients to more complex congenital malformations than patients with an isolated cloaca. The posterior sagittal approach has been used effectively for repair of these congenital malformations.