Sacral agenesis is a rare congenital vertebral anomaly which is often associated with bladder, bowel and lower limb neuromuscular dysfunction. An isolated bladder involvement is quite exceptional. Here we are reporting one such neglected case with isolated bladder involvement leading to chronic kidney disease. Submit Manuscript | http://medcraveonline.com Urol Nephrol Open Access J 2015, 2(2): 00032 but usually it is associated with other abnormalities, such as anorectal malformation and mal development of the bones or joints of the lower limb [3]. Voiding dysfunction and recurrent urinary tract infection are common urological presentation [2]. However, kidney damage could occur if diagnosis is delayed and not intervened in time. Here we present a neglected case of sacral agenesis with neurogenic bladder leading to chronic kidney disease.