Prune belly syndrome is characterized by a triad of abnormalities, including an absence or deficiency of abdominal wall musculature, cryptorchism, and anomalies of the urinary tract. The characteristic deficiency of the abdominal wall musculature was first described by Frohlich et al.1 in 1839. Parker2 first reported the association of the genitourinary anomalies with the deficient abdominal musculature. The term “prune belly syndrome” was coined for this complex by Osler in 1901.3 Eagle and Barrett,4 in 1950, further defined the triad of absent abdominal wall musculature, undescended testes, and urinary tract abnormalities. The incidence of prune belly syndrome is estimated to be 1 in 27,000 to one in 40,000 live births.5 7 This syndrome occurs almost exclusively in boys. In females, it is extremely rare, with less than 50 cases described in the world literature.7 , 8 It is also known as pseudo–prune belly syndrome and is characterized by hypoplastic abdominal musculature and urinary tract and genital anomalies, most commonly bicornuate uterus and vaginal atresia.8