Noonan syndrome is an autosomal dominant genetic disorder with multisystem involvement inclusive of distinctive facial features that rather go unnoticed. The possible correlation of the oral manifestations with the various other systemic findings will enable a multidisciplinary approach and timely management. We describe an exceptional case of Noonan syndrome with typical facial characteristics, skin and oral manifestations with Keratocystic odontogenic tumor of mandible and predilection to Neurofibromatosis. The article also aims to put forth our clinical strategy of management of a patient diagnosed with this syndrome.