Introduction. The objective of this study was to verify the long-term outcome of transtrochanteric anterior rotational osteotomy (ARO) for osteonecrosis of the femoral head (ONFH) in young patients with systemic lupus erythematosus (SLE). Methods. Consecutive series of 21 symptomatic ONFH patients with SLE (33 hips), aged 20 to 40 years, underwent ARO between 1980 and 1988. We reviewed the cases of 16 patients (25 hips), which represents a 76% rate of follow-up. Patients included 4 men and 12 women who had a mean age of 29 years at the time of surgery. A Kaplan-Meier curve was used for the survivorship analysis of ARO. Patients with surviving hips were evaluated by the modified Oxford hip score and the Medical Outcomes Study Short Form 36 (SF-36). Results. Twelve hips in 8 patients survived at the final follow-up. The average length of surviving was 25 years (range, 20 to 27 years). Three patients (6 hips) had died of unrelated causes without any conversion at the mean time of 9 years after ARO. Based on Kaplan-Meier analysis with the end point defined as any conversion, the survival rate at 25 years was 73.7% (95% confidence interval, 53.9 to 93.5%). Based on the classification of the modified Oxford hip score, 5 hips were classified as excellent, 2 hips were good, and the remaining 5 hips were fair. The average SF-36 physical component summary score was 34 points and the average mental component summary score was 46 points. The physical component summary scores of 3 patients (53.0, 56.6, 57.1) exceeded the level of the Japanese population norm. Conclusion. In ONFH patients with SLE, ARO achieved a 73.7% survival rate at 25 years