Osteopetrosis is a group of rare hereditary skeletal disorders with variable clinical features which are characterized by a marked increase in bone density and reduction of marrow spaces. This disease has been generally reported in three clinical forms: severe malignant infantile autosomal recessive inheritance form; intermediate mild autosomal recessive form with clinical manifestations similar to malignant form and having the lowest incidence rate; and benign/adult osteopetrosis with autosomal dominant inheritance and with fewer symptoms. This report presents a case of osteopetrosis in a 27 year old Syrian female patient who was referred by her physician for dental checkup. The best therapeutic approach for this patient was preventive management and maintenance of good oral hygiene by regular brushing of her teeth twice a day and intake of a balanced diet and reduce the amount of sugar and carbohydrate to prevent dental caries.