Congenital nasal abnormalities are very rare in the literature. There are 3 reported cases of isolated partial lower lateral cartilage defects. In this article, we report the case of a patient with congenital complete absence of the lower lateral cartilage. The patient had severe external nasal valve dysfunction and a concavity of the alar vault, even in the resting position. The defect was reconstructed using resected and reshaped nasal dorsal hump material. At the end of a 12-month follow-up period, the patient was satisfied with the functional and aesthetic results of the operation. No external or internal nasal valve collapse occurred during inspiration. Such developmental abnormalities may be syndromic and require special attention for reconstruction.