Pagets disease is a chronic skeletal disease of middle and later life, beginning insidiously and characterized by progressive structural changes and typical deformities occuring in the long bones, pelvis, and cranium. The occurence of Pagets disease is extremely rare in Korea. We have experienced a case of Pagets disease of bone in 67 year-old female affecting mainly the ilium and skull, and we report the case with brief review of the literatures.