Supernumerary nostril, also known as triple nostril or accessory nostril, is one of the extremely rare congenital nasal deformities which includes an additional nostril. Since Lindsey reported the first case of a supernumerary nostril, only 34 cases of supernumerary nostril have been reported world widely. And there was no any domestic case. In the present case, we described a case of supernumerary nostril and reviewed all the literature cases of supernumerary nostril.

A 10-month-old female patient visited to the authors with an additional nostril located above her right nostril, which had been present since birth. Antenatal history was uneventful and the infant's birth was normal. On physical examination there were no other abnormalities and additional nostril was communicating with ipsilateral normal nasal cavity. We performed fistulectomy and local flap for the correction.

After 7 months postoperatively, the patient was doing well. The functional outcome was excellent and the cosmetic result was satisfactory. During the long term follow-up for 8 years, there were no specific problems.

In supernumerary nostril, preoperative evaluation of other abnormalities is very important and we advocate that corrective surgery can be performed at an early age for patient's psychosocial development.