Among the cranio-facial anomalies involving the orbit, Paget's disease is of particular interest due to its progressive evolution and clinico-pathological characteristics. In this article a case of Paget's disease (juvenile type) with orbital expansion in a young woman of 27 years is presented. The onset of clinical symptoms were recorded at the age of 15 years progressing to optic atrophy and blindness in one eye and visual deterioration on the other. The peculiarity of this case lies on the early onset of clinical symptoms due to elective in volvement of the skull and sparing of the facial bones. The surgical decompression (removal of the roof of the orbit and upper wall of the stenosed optic canal) was successful in controlling the evolution to optic nerve atrophy in the seeing eye.