Osteopetrosis is one cause of diffuse osteosclerosis and bone fragility due to abnormal remodelling rate and balance. It may cause some serious skeletal abnormalities, with particular respect to long bones, and oral complications such as osteomyelitis and exposed necrotic bone. Orthopaedists and dentists should be aware of patients with the disease because of its effect on osteoclast function, which results in impaired wound healing. The purpose of this paper is to review the aetiology, pathogenesis and bone remodelling of osteopetrosis leading to some guidance for orthopaedic surgeons and dentists on the management of patients with osteopetrosis, with particular attention to fractures.