Abstract

Pyknodysostosis is a rare autosomal recessive disorder. This disorder has defective gene that codes for enzyme Cathepsin K which inhibits the normal osteoclastic function, making the bones abnormally dense and brittle. Extraoral manifestation includes increased bone density, skull deformities with delayed suture closure, proptosis, and frontal bossing. Intraoral features include anterior open bite, posterior crossbite, crowding of teeth, poor oral hygiene, and periodontal disease. Pyknodysostosis is a rare disorder of bone showing various craniofacial features. These patients usually have normal lifespan. Thorough history and clinical examination aids in diagnosis. There is increased risk of mandibular fractures and osteomyelitis following extraction. Managing with minimally invasive procedures and proper follow-up is required to prevent its complications due to invasive dental treatment.