Aim: This is a case report of a 16-year-old girl visiting MR Ambedkar Dental College and Hospital (Department of Pedodontics and Preventive Dentistry) for dental treatment. Background: Osteopetrosis acroosteolytica or Toulouse-Lautrec syndrome or pyknodysostosis is a rare autosomal recessive bone dysplasia, characterized by osteosclerosis, and short stature. Montanari described a patient with an unusual variation of achondroplasia, which in retrospect was the first case of pyknodysostosis to be reported.1Case description: A 16-year-old girl reported to the Department of Pediatric and Preventive Dentistry with a chief complaint of pain in the lower left back region of the jaw since past 2 weeks. Conclusion: Pyknodysostosis is a rare condition that is diagnosed basically on its clinical and radiographic features. Clinical significance: It is important to recognize these features so that correct diagnosis can be made. This allows the treatment and prevention of future complications and ensures a better quality of life to the patient.
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