Skeletal surveys were performed on a 38-year-old Japanese mother, her son and daughter. The radiographs of both children showed characteristic features of osteopathia striata. However, in the mother, the skull, mandible, and lower extremities were homogeneously sclerotic with no evidence of a striated pattern of sclerosis in her skeleton. Additional features of striated sclerosis of the mandible in patients with osteopathia striata are discussed.