Osteopetrosis is a rare skeletal disease of unknown etiology. Osteomyelitic changes in the jaw bones are frequently seen in this disease, especially in the mandible and may follow upon tooth extraction. A case is reported of a 31-year-old male who was seen because of intraorally exposed bony spicule and chronic dull pain. Radiographic and haematological investigations led to the diagnosis of osteopeirosis with osteomyelitis. The striking radiologic findings were pathologic mandibular fracture and generalized skeletal thickening. The present case could be grouped under the benign form, type II osteopetrosis. With the conservative and minimally invasive modes of treatment including administration of penicillin G sodium, curettage and closed reduction, we could successfully manage the osteomyelitis and pathologic fracture associated osteopetrosis.