Osteopathia striata with cranial sclerosis is a very rare sclerosing bone dysplasia that is characterized by the radiological appearance of longitudinal striations at the metaphysis of the long bones associated with cranial sclerosis. Here, we report a 10-month-old female infant and a 31-year-old male, who were diagnosed incidentally by showing longitudinal sclerotic lines symmetrically in the long bone ends and basal sclerosis of the cranium. The diagnostic linear striation was found not only in the long bones but also in the ribs and phalanges, which are very rare sites for striation. We discuss the radiology findings of osteopathia striata with the possible complication of cranial sclerosis.