Hernia through a defect of the broad ligament is extremely rare in children. These defects can result from a developmental defect or a spontaneous rupture of cystic formations remnants of mesonephric ducts or Müller. Genital anomalies associated with unilateral renal agenesis are more common in females. We report the case of a 13 months girl allowed for assessment of recurrent urinary tract infection; abdominal examination did not objectified palpable mass, the external genitalia were without abnormalities. Abdominal ultrasound revealed a left renal space is empty with a retrovesical cyst. Cystography was requested that objectified a large pelvic cystic mass retrovesical communicating with the bladder, there was also a left vesicoureteral reflux. Uro- MRI showed a cystic formation retrovesical communicating with the bladder, the right pelvic kidney; uterus is normal size for age. DMSA scintigraphy confirmed the absence of the left kidney with the right kidney that ensures 100 % of total renal function. To surgical exploration we found a hernia of the bladder through the left broad ligament, the uterus was dislocated on the right side; left ovary was hypoplasic; the ipsilateral ureter was blind with renal agenesis, we performed by reduction of the bladder then closing the hernial orifice, dissection of the ureter with its ligation and section at the vesical stoma. The postoperative course was uneventful. Evolution is favorable. This observation illustrates a hernia of the bladder through the broad ligament associated with ovarian hypoplasia, renal agenesis and ipsilateral ureter blind ending; this association was not described to our knowledge in the literature.