Posterior urethral valve (PUV) commonly presents during infancy. We report a case of a 5-year-old boy with Down syndrome who was diagnosed to have PUV in late childhood. He presented with recurrent left epididymo-orchitis, an uncommon but recognized com-plication of PUV. Our aim is to discuss this rare and delayed presentation of PUV in par-ticular amongst children with Down syndrome, method of diagnosis and complication of the disease if left untreated.