[Osteopathia striata with cranial sclerosis]

Osteopathia striata with cranial sclerosis is a rare disease: fewer than 100 cases have been reported. The radiologic findings of osteopathia striata are characteristic, and once they are identified, they lead to the correct diagnosis. Longitudinal sclerotic striation in long bones and osteosclerosis in facial bones should raise suspicion of osteopathia striata with cranial sclerosis. This is not a serious disease, although it is often associated with other kinds of disorders and extraskeletal malformations that can affect the prognosis. Involvement of cranial and facial bones can lead to facial deformity and marked functional incapacity when the cranial nerves are affected. We present a case of osteopathia striata with cranial sclerosis discovered incidentally in a young woman studied for clinical manifestations unrelated to this disease.