Diastrophic dysplasia results in severe disproportionate growth failure, generalized joint dysplasia, and early osteoarthrosis of the hips. A total hip arthroplasty is often necessary in patients afflicted with diastrophic dysplasia by time they reach early middle age. During 1983-1988, total hip arthroplasties were performed on six women and four men (15 hips) with diastrophic dysplasia at the Orthopaedic Hospital of the Invalid Foundation. The mean age of the patients at the time of operation was 37 years and the mean height was 133 cm. A Lord endoprosthesis was used in nine hips and a Biomet endoprosthesis in six. Cementless fixation was used in all cases. Autogenous bone grafting to the acetabulum was performed in six hips. Simultaneous corrective osteotomy of the proximal femur and transposition of the greater trochanter was performed in three cases. Soft tissue release and tenotomies were performed in 10 hips. The average follow-up period was 5 years. Overall clinical results were good, with marked relief of pain and improvement of hip joint mobility. Aseptic loosening of the acetabular component was noted in two hips. As for complications, two femoral nerve paresis and two perioperative fractures of the proximal femur occurred, which all healed.