Osteogenesis imperfecta is a heterogeneous genetically disorder and is inherited as an autosomal dominant or outosomal recessive trait. The primary defect lies in the mutation of genes that encode type 1 collagen. Organs bearing connective tissue such as bone, sclera, inner ear, skin, ligaments, tendons, and fasciae are involved. With respect to clinical, biochemical and inheritance characteristics, this disorder is divided into 4 groups (Ol l, Ol ll, Ol lll, and Ol lV). The clinical feature consists of mainly fragile bones with or without blue sclerae and deafness. In report an 8- year old boy is presented with or a pedigree suggesting an autosomal recessive type of inheritance in whom with respect to clinical feature, osteogenesis type llB is diagnosed.