a b s t r a c tThis case describes the challenges associated with total hip arthroplasty in a patient with unique anatomy, including developmental dysplasia of the hip, pelvic dysmorphism, and unilateral sacroiliac joint autofusion. A 30-year-old female, with a history of developmental dysplasia of the hip treated with presumed pelvic osteotomy complicated by postoperative infection, presented with hip pain refractory to conservative management. Radiographic studies demonstrated a 10-cm leg length discrepancy, 20 of acetabular retroversion, severe hemipelvic dysmorphism, ipsilateral sacroiliac joint autofusion, and significant femoral head dysplasia. Total hip arthroplasty was performed using a revision acetabular component and modular femoral component, resulting in improvement in the postoperative leg length discrepancy. There were no neurovascular or other perioperative complications, and the patient was ambulating without pain or assistive devices at 1-year follow-up.