Sacral agenesis is an uncommon condition associated with familial diabetes mellitus and complicated pregnancy. The condition is characterized by gluteal skin dimpling, loss of gluteal fold, myelomeningocele, sacral lipoma, as well as numerous orthopedic, anorectal and other widespread anomalies. The motor nerve deficit, for example poor anal sphincter tone, is more pronounced than the sensory nerve deficit, for example perianal anesthesia. Diagnosis is made by anteroposterior and lateral spinal x-rays. All of our patients had bladder or external sphincter dysfunction, while 91 per cent had vesicoureteral reflux. Early diagnosis and conservative management with intermittent catheterization and uropharmacological agents are now advocated.