Objective:

To describe the case of a female patient with Prune Belly syndrome, which is rare condition.

Case presentation:

17-year-old female patient with a diagnosis of Prune Belly syndrome since birth, with defects of abdominal muscles with loose and wrinkled skin, and anorectal malformation. Later, urinary tract defects were detected that caused recurrent urinary infections. In adolescence, little development of external genitalia, didelphys uterus and thoracoabdominal scoliosis were detected.

Conclusions:

Prune Belly syndrome in women accounts for less than 5% of cases. It is important to recognize the clinical findings to make an early diagnosis of the associated complications. Management must be multidisciplinary, which should include the participation of specialists in Genetics.