Osteopetrosis is a rare genetic disorder of bone, characterized by impaired bone remodeling. Hyperostosis occurs due to lack of resorption of immature bone by osteoclasts. Abnormal remodeling of primary, woven bone to lamellar bone results in a brittle bone. In most ethnic groups, osteopetrosis occurs where consanguinity is common. The purpose of this article is to present a case of osteopetrosis and comprehensive review of its classifi cation, clinical description, diagnosis, and management.