Prune Belly syndrome (PBS) is an extremely rare anatomo-radiological syndrome that combines aplasia of the muscles of the
anterior abdominal wall, dilatations of the urinary tract and testicular malformations, thus forming the classic triad of the
syndrome. Up to 75% of patients with PBS have pulmonary, skeletal, cardiac, and gastrointestinal malformations. We report
the case of a full-term male infant with no particular pathological history admitted for etiological work-up of bilateral
ureterohydronephrosis diagnosed antenatally with a distended, prune-shaped abdomen with bilateral testicular ectopia on
clinical examination. The outcome can be very variable, ranging from stillbirth due to major renal and respiratory dysplasia to
a practically normal child, all of which explains the great diversity of opinions on the attitude to adopt when faced with this
syndrome.