Pycnodysostosis (PDO) is a human autosomal recessive genetic disorder characterized by osteosclerosis of the skeleton, short statue, and severe bone fragility. This unusual syndrome usually involved typical craniomaxillofacial deformities which were composed with hypoplastic midface, collapsed palate, open mouth posture, beaked nose, micrognathia, anterior crossbite, dental crowding and over-retained deciduous teeth. Early diagnosis and intervention are mandatory. The present study reported staged approach case for a PDO patient to correct his dentofacial deformity.