Abstract
A 17 year old male patient presented with bilateral preauricular sinus, right sided second branchial cleft sinus and bilateral hearing deficit. He has previous history of right congenital cataract surgery and right dacryocystorhinostomy at the age of 8 year. He was operated for branchial sinus. No family history with similar complaint was observed. According to all these features this case was the variant of branchio-oto-renal spectrum disorder with de novo mutation. According to literature search this will be a unique and rare case report of BORSD published till date in India.