We report a case of a rare congenital nasal malformation. A young male patient was seen with a unilateral duplication of the left nostril. According to the morphogenetic classification, described by van der Meulen, this nasal malformation could be classified as a type IV nasal dysplasia. The other observed abnormalities that were seen were part of the CHARGE association, which is defined as coloboma of the iris, heart deformities, choanal atresia, retarded growth, genital and ear deformities. The patient underwent a surgical correction of the nose using an L-approach, yielding a satisfactory result 1 year postoperatively. Details of the case and a review of the scarce literature are presented.