Abstract: Branchio-oto-renal spectrum disorders (BORSD) is a complex and rare autosomal dominant inherited deafness syndrome. Branchio-oto-renal syndrome (BORS) is the main clinical condition. Branchio-oto syndrome (BOS) can be called if there is no obvious renal abnormality. A case of BOS associated with nephrolithiasis is reported.

Key words: branchio-oto syndrome, congenital malformation, hearing loss, branchial cleft fistula