Accessory nostril is a very rare congenital anomaly with an unknown etiology also known as supernumerary nostril. A few accessory nostrils have been reported up to the present time, and extremely rare cases located on columella. A newborn infant with respiratory distress was referred to our hospital. The authors observed that accessory nasal nostril is not related to normal nasal cavity on the median line of columella. In this article, the authors reported accessory nostril case in newborn and review the literature.