Background: Marble bone disease is an inherited rare skeletal condition characterized by increased bone density on radiographs and involves not only skeletal system but also hematological,neurological systems as well as dental problems which is inherited either as autosomal dominant or autosomal recessive. Incidence 1 in 200,00LB and 1 in 2,00,000 LB respectively. These conditions vary greatly in presentation and severity ranging from neonatal onset with life threatening complications such as bone marrow failure. We describe a case of 8-year-old female with the typical radiographic and clinical features which doesnot require genetic testing for the diagnosis.