Osteopetrosis is a rare hereditary skeletal disorder characterized by a marked increase in bone density resulting from the abnormality in bone remodeling. Radiographically, there are widespread increase in skeletal density and the loss of distinction between cortical and cancellous bone. In addition to exhibiting widespread osteosclerosis, osteopetrosis may manifest hematologic and neurologic disorders. Clinically, these patients have susceptibility to anemia, infection, and so on. There is no specific therapy for osteopetrosis. Supportive therapy such as transfusion and control of infections could be done. We report a case of the osteopetrosis developing osteomyelitis of the maxilla with suppuration and facial swelling.