ABSTRACT
This report describes a case of osteopathia striata with cranial sclerosis [OS-CS] in a Bedouin girl. This patient to our knowledge is the first case to be reported in KMGC, throwing light on the clinical and radiological findings. Clinical examination, skeletal survey, echocardiography, ultrasonography and chromosomal study. The patient had craniofacial dysmorphic features including overgrowth of the craniofacial bones which is characteristic of the disorder, developmental delay, hearing impairment, congenital heart [VSD] and she had also repeated attacks of seizures. Radiographic findings included marked ossification and sclerosis of the base of the skull and metaphyseal widening of long bones with linear striations. In the few reported cases of OS-CS all over the world, our case could have the typical form of the syndrome with dominant inheritance and the first case to be described in Kuwait