Extrophy of the urinary bladder is a rare congenital anomaly first described in ancient times, and which C.H. Mayo (1919) called “the most serious and troublesome of all human defects to contend with”. The condition occurs once in every 30,000 or 40,000 live births, and is more common in males than females. It poses a challenging and fascinating problem in management for the surgeon and has accumulated an extensive literature. The purpose of this paper is to present a brief review of the disorder with emphasis upon treatment.