A wide spectrum of renal, ureteral, and urethral abnormalities has been reported in a patient born with Prune belly syndrome (PBS). The obstruction in the urinary tract can be found anywhere from uppermost (pelviureteral junction) to lowermost (prostatic membranous urethra) urinary tract. The mesenchymal developmental arrest is the main embryological factor responsible for the major features of the syndrome like urethral abnormalities or gastrointestinal abnormalities. PBS patients with urethral obstruction commonly had a patent urachus, because of which such patients survived, early death usually occurred in those patients who did not had a patent urachus. We are reporting an interesting case of prune belly syndrome with superior vesical fissure (an exstrophy variant). To the best of our knowledge this was first reported case of association of two congenital anomalies having common embryological defect process. Presence of superior vesical fissure gives a new insight about the benefits gained by the child for surviving with such fatal congenital anomalies.
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