INTRODUCTION: Caudal regression syndrome (CRS) is a rare neural tube defect affecting terminal spinal segment. It manifests as total neurological deficit in the lower limbs and loss of bladder and bowel control. It may be associated with other structural anomalies so that patients are more likely to present first to the pediatric surgeon, the urologist, the orthopedic surgeon or neurosurgeon, than to the pediatric gastroenterologist. Delayed diagnosis increases the risk of renal impairment, recurrent urinary tract infections, and fecal incontinence. CASE REPORT: A 27 month-old boy was referred to our pediatric GI department for a several-month history of constipation. He was born to a mother with type 2 diabetes and had a history of hypocalcemic seizure at 10 days of age, when initial workup included PTH, TFT and CATCH 22 FISH was unremarkable. He was also diagnosed with congenital hearing impairment of both when newborn hearing screening test was performed. The mother of the patient noticed that his constipation had started since infancy. She also complained that it was hard to control his diarrhea when she tried small amounts of laxative. His voiding stream was not considered to be weak and dribbling of urine was sometimes noticed. On examination, he had low set ears with hearing aids and small buttocks with dimples at the lateral aspects of the upper buttocks. However, he was active and had passed normal mental milestones except a delay of language development. He showed no motor deficit of his lower limbs and had sensation in the lower limbs and perineum without any gross deformities. X-ray of the abdomen revealed absence of the distal sacrum and the coccyx. A voiding cystourethrogram showed poor bladder filling with no reflux. MRI of the spine identified abrupt termination of the conus medullaris at the level of T12~L1 and thickened filum terminale. Abdomen and kidney ultrasonography, echocardiography and Brain MRI are normal CONCLUSION: This is a unique case report there was a congenital sensory-neural hearing loss along with the CRS. We present this case to highlight the fact that spinal dysraphism should be kept in the differential diagnosis when confronting the patient with chronic constipation and congenital anomalies even though showing no neurologic deficit.