Congenital nasal anomalies are rare disorders. An 11-year-old girl presented to our clinic with a left sided nasal obstruction and chronic nasal discharge since birth. Clinical evaluation revealed membranous septa which divided the left nasal cavity into two blind pouch and left sided choanal atresia with alar cartilage asymmetry. This congenital anomaly was not defined in the English literature. The membranous septa in the left nasal cavity were excised, alar reconstruction were performed and the choanal atresia were repaired by a transnasal endoscopic approach. In this article we reported a case of membranous septa which divided the left nasal cavity into two blind pouch associated with unilateral choanal atresia and alar cartilage asymmetry. Also the nasal anomalies and associated disorders in the literature were reviewed. © 2009 Elsevier Ireland Ltd. All rights reserved.