Barakat syndrome, also known as HDR syndrome is an autosomal dominant disorder characterized by hypoparathyroidism, sensorineural deafness, and renal dysplasia caused by mutation of the GATA3 gene.The syndrome was first noted in siblings with hypocalcemia and proteinuria. It is a genetic developmental disorder with clinical diversity; affected people usually present with hearing loss, tetany, seizures, numbness and renal disease at any age. Hearing loss is usually bilateral and may range from mild to severe. Renal disease mainly includes renal-dysplasia, nephrotic syndrome, cystic kidney disease. Other reported features are intellectual disability, polycystic ovaries, distinct facial characteristics, ischemic stroke and retinitis pigmentosa. Treatment is symptomatic and prognosis depends upon the severity of renal disease.