A 2-year-old boy with bilateral cervical fistulae, bilateral pre-auricular fistulae and hearing impairment was reported. His father had the same symptoms and his elder sister had also bilateral pre-auricular fistulae. These symptoms were considered to fit in Branchio-Oto-Renal (BOR) syndrome defined by Melnick et al, although renal abnormality was not demonstrated in this case. This is the forth case of BOR syndrome reported in Japan.
