Bilateral, uncemented hip replacements were performed on a 45-year-old woman with autosomal dominant osteopetrosis. The hips showed degenerative changes and protrusio acetabuli. Difficulties were encountered especially during preparation of the femoral canal. At ten-year follow-up she has an excellent clinical and radiological result with no sign of osteolysis. Uncemented hip replacement, while technically demanding, can be successful in the intermediate term for patients with this condition.