Osteopetrosis is a rare disorder characterized by symmetrical systemic sclerosisof skeletal bone. Osteomyelitis of the jaws is a frequent complication in the oral and maxillofacial region. We report here a case of osteomyelitis of the mandible that occurred 2 years after tooth extraction in a patient with osteopetrosis. The osteomyelitis was successfully treated by removal of the necrotic bone and granulation tissue, hyperbaric oxygen therapy, and antibiotic therapy.