We report a case of a male newborn with asymmetrical epispadic diphallia and congenital urethral fistula, and exomphalos minor. A segment of bowel was attached in the region of the pubis; with no communication to the underlying bowel. This was excised; histological examination revealed this to be a colonic remnant. The excision of the examphalos minor and approximation of the pubis and the rectus sheath over the bladder was also done; urethroplasty is planned at a later date. A comprehensive review of the available literature revealed only eight such cases before this; the possible embryology is discussed.
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