Introduction: Congenital anomalies of the nose are rare and occur in 1 of 20,000 to 1 of 40,000 newborns. Congenital absence as an isolated deformity of the lower lateral cartilage (LLC) is extremely rare with only two cases described in the current literature. In one of the described cases, there was a total absence of the LLC, whereas in the other case, the absence was only partial.