Avascular necrosis of the femoral head is 1 of the skeletal manifestations of Gaucher's disease, for which hip arthroplasties are performed in many cases. Because many patients are young, total hip arthroplasty in this condition has been reported to result in aseptic loosening of the components soon after operation. Osteotomy can prevent such a problem. The authors present a successful case of a 17-year-old boy who underwent transtrochanteric anterior rotational osteotomy for avascular necrosis of the femoral head in Gaucher's disease. This procedure is an effective and safe surgical treatment for this disease if performed before the onset of significant femoral head collapse or acetabular deformity.