The nose is a prominent feature of the human face. Congenital malformations of the nose, whether functional or anatomic, affect the physiologic and psychologic wellness of children who have these anomalies. Congenital nasal abnormalities may be overt or subtle and can occasionally cause life-threatening emergencies at birth. A discussion of nasal embryology and development provides the basis for the discussion of some of the important congenital abnormalities seen in clinical practice. The final portion of the article is devoted to several of the more common syndromes in which nasal abnormalities are encountered.