Osteopetrosis is a descriptive term that refers to a group of rare hereditary disorders of the skeleton characterized by increased density of the bones. It was first described in 1904 by Albers-Schonberg, a German radiologist. This disease varies greatly in its presentation and severity ranging from neonatal onset with life threatening conditions such as bone marrow failure to incidental finding of osteopetrosis on radiographs. Dental abnormality may be attributed to pathological changes in the bone. Dentists should be aware of patients with the disease because of its effect on osteoclast function which results in impaired wound healing. This paper presents the case of a five-year-old girl exhibiting oligodontia associated with osteopetrosis.