Six cases of Bardet-Biedl syndrome are presented. Common findings in this syndrome include mental retardation, pigmentary retinopathy, obesity, and hypogonadism. Uremia is stated to be the cause of death in more than one-third of reported cases. In almost all cases, cystic spaces communicating with the collecting system are demonstrated by excretory urography. The origin of these spaces is not understood. Assessment of the urinary tract by excretory urography and probably by catheter cystography should be undertaken in all patients with this syndrome to assist in proper management.