Until the recent past, patients born with congenital absence of the abdominal musculature, and associated deformity of the urinary collecting system, have often had an unhappy life and an early death. Twelve years ago, a treatment protocol was devised in our institution which included the following major steps: 1) bilateral tubeless pyelostomy immediately after birth; 2) serial evaluation of renal status during the first year of life; 3) laparotomy at one year of age with orchiopexy, reconstruction of the urinary collecting system and reconstruction of the abdominal wall; 4) serial electromyographic evaluation of the growth and development of the lateral abdominal musculature; 5) serial studies of renal function; 6) secondary revision of the abdominal wall and of the urinary drainage system as needed. Nine patients so treated have been followed from the third through the twelfth years of life. All are alive and growing well. Four patients had chronic urinary infection treated with long-term antibiotics; three of these have required a second surgical correction of some aspect of the urinary drainage system. Three patients have required additional surgery on the abdominal wall, but have recaptured enough function so that corsets and other devices have not been necessary. Electromyographic studies show good growth and development of some aspects of the lateral abdominal musculature which has been used in the repair. The outcome in these patients is highly encouraging for this constellation of anomalies. The cause remains controversial.