Acquired hemophilia A (AHA) is a very rare disease of idiopathic type, or secondary to neoplastic disease, the use of some drugs, or pregnancy. Owing to the rarity of the disease, no comparative clinical studies are currently available on the results of different intraoperative and postoperative pharmacological preventive strategies in arthroplasty surgery. We report the first case of a total hip replacement procedure for a fracture of the femoral neck performed in a patient affected by AHA. Treatment with recombinant factor VII was administered, at the dosage of 170 μg/kg preoperatively and continued postoperatively at 120-90 μg/kg every 2-6 h. The outcome described supports the use of the protocol adopted, demonstrating that the treatment duration was well adapted to this highly invasive surgical procedure.