Pyknodysostosis is a rare skeletal disease considered to be a variant of osteopetrosis. Clinical features of pyknodysostosis include dysplasia of the skull with frontal and parietal bossing, dysplasia of the clavicles, and partial or total aplasia of the terminal phalanges. The tips of the fingers are unusually club shaped, with short, irregular terminal digits. Often the paranasal sinuses are hypoplastic or absent, and the fontanelles are patent and palpable. Pathologic fractures commonly occur in childhood because of the dense, fragile bones. Pectus excavatum, bowing of long bones, bilateral genu valgum, clavicular hypoplasia, as well as kyphosis and scoliosis, are additional orthopedic presentations of this disease. Morphologically, the skull appears disproportionately, large in comparison with the rest of the body, but the face is small because of the hypoplastic development of the facial bones. The eyes appear exophthalmic, and the nose has been described as a parrot's beak. Histologically, pyknodysostosis shows continuous endosteal bone formation without concomitant resorption and modeling. Although active endosteal bone formation usually produces a reduction in bone marrow volume, pyknodysostosis is not characterized by severe anemia. This suggests active medullary hematopoiesis. Prevalent oral features have been reported by Yamada et al. 3 , and include premature eruption of teeth, shortness of tooth roots, narrowing of pulp cavities, hypodontia, enamel hypoplasia, malocclusion and malposition of teeth, a double row of deciduous teeth in both the maxilla and the mandible, severe caries, macroglossia, a grooved palate, obtuse mandibular gonial angles, and generalized micrognathia 3 . Osteomyelitis of the jaws and recurrent fractures of long bones are characteristic complication of pyknodysostosis. 1,4,5 Green and Rowe 6 described a case of osteomyelitis occurring in a patient with pyknodysostosis after extraction of a mandibular first molar. Reconstruction was attempted using a titanium tray and an iliac crest bone graft. The reconstruction failed subsequent to infection. There has been no documented case of successful mandibular reconstruction in patients with pyknodysostosis. We report the management of osteomyelitis in a patient with pyknodysostosis and the early results of mandibular reconstruction.