Noonan syndrome (NS) is a common congenital syndrome characterized by multiple anomalies commonly observed in children. In this article, we describe a case of a patient with congenital heart disease, severe mitral regurgitation, and Nonaan syndrome presented with left maxillary swelling and pain, which was treated by complete surgical excision of the left Maxillary Odontoma. Based on this case, we conclude that numerous oral abnormalities may be related to NS and thus necessitate interdisciplinary treatment planning and prompt therapy. The importance of including oral manifestations as a scoring criterion in diagnosing NS cannot be overstated, as the significance of oral findings in NS has largely been overlooked.
