Cloacal dysgenesis with urethral, vaginal outlet, and anal agenesis and functioning internal genitourinary excretion

Cloacal dysgenesis, a rare anomaly, occurs in female infants when the Müllerian system differentiation fails to delineate the genitourinary and lower alimentary tracts. Embryologically, the dating of the lesion can be traced to a point between 4 and 5 weeks into gestation. The exact mechanism is usually not known. We observed an infant with cloacal dysgenesis involving urethral, anorectal, and vaginal outlet obstruction. Minimal hydronephrosis, absence of hydroureters, and pulmonary hypoplasia are all related to the cloacal dysgenesis.