Urethral agenesis with complete absence of the urinary meatus is a very rare anomaly which comes in two forms, either with normal phallus or in combination with penile agenesis (Figs. 23.1 and 23.2). It is a very rare reported anomaly as most cases die intrauterine; combined bladder and urethral agenesis is also an extremely rare anomaly with only 22 live births have been reported of the 60 known cases. Few cases have been reported in females as this condition is mostly reported in boys [2].

Penile agenesis complicated by Potter sequences with urethral agenesis should be differentiated from those with ectopic urethral opening. This anomaly is usually associated with anorectal malformations and limb anomalies, and it may represent the spectrum of urorectal septum malformations, which lead to several different types of fistulas between urinary system and rectum, or urethral agenesis, specially with partial urorectal septum malformation sequence [3]. Embryologically the urorectal septum, grows downward from the ridge separating the allantois from the cloacal opening of the intestine and ultimately fuses with the cloacal membrane and divides it into an anal and a urogenital part, where the dorsal part of the cloaca forms the rectum, and the anterior part of the urogenital sinus and bladder [4].