A 30-year-old male patient presented to our hospital with pain in the left hip region, reduced leg mobility due to theleft hip PJI with two fistulas in the upper lateral and anterior thigh region. Collecting anamnesis, the patient had a left distal femoral metaphyseal fracture due to a fall at the age of 10. At that time, X-rays showed dysplastic bone lesions of the left femur and the patient was diagnosed with monostotic FD. The patient had distal femoral osteosynthesis with a dynamic compression plate. The fracture healed without any complications. The plate was evacuated 1 year later and the dysplastic bone lesions were sealed with a bone allotransplant. Ten years later, the patient had a pathological left femoral neck fracture without any trauma. Osteosynthesis with intramedullary femoral nail was performed in another hospital. Six weeks after the operation, the patient developed growing pain and swelling in the left hip and distal thigh region. Septic arthritis of the left hip joint was diagnosed. The intramedullary nail was removed, femoral head resected, and the patient received systemic antibiotic therapy for 6 weeks. Afterward, debridement of the distal femoral metaphysis was performed, and bony cavities were sealed with bone graft substitute made of calcium sulfate. After 8 weeks, there were no signs of active infection and the patient over went total left hip arthroplasty with anuncemented prosthesis. Three years later, the patient developed a PJI. Local debridement, removal of the prosthesis, and placement of cement spacer incorporating gentamicin () were performed in combination with systemic antibiotic therapy. Six weeks later, the spacer was removed and these conduncemented total hip arthroplasty was performed. As a complication, the patient developed lymphedema of the left leg. One year later, the patient had a repeated PJI which was treated with debridement, retention of the prosthesis, and systemic antibiotic therapy. Six months later, the patient developed deep vein thrombosis of the right axillary vein, which was treated with anticoagulant therapy. He was diagnosed with antiphospholipid syndrome and thoracic outlet syndrome. One year later, the patient developed posterior displacement of the femoral component in the left hip joint twice with a 1-month interval and both were reduced in a closed manner. One month later, he developed a repeated PJI which was treated with debridement, drainage, and systemic antibiotic therapy. Two fistulas opened in the left thigh region and the patient was transferred to our hospital. The patient had pain during motion in the left hip and moderate decrease in the range of motion. Routine analyses showed leukocytosis (15.90 × 109L) and increased C-reactive protein (67.1mg/L). Radiographs of the left hip showed osteolytic zones around the acetabular and femoral components with sclerotic deformation of the distal femoral metaepiphysis ( and ). Our plan was a two-stage exchange hip revision arthroplasty by performing removal of the prosthesis, implantation of an antibiotic-impregnated articulating cement spacer, and after 6 weeks of systemic antibiotic therapy– implantation of a distally-interlocked modular femoral reconstruction prosthesis due to the severe bone defects in the left femur. The operative approach was through the previous operation scar of anterolateral approach– the third fistula was found underneath the scar tissue which was connected to the greater trochanter region. Tissue samples were taken for microbiological examination. Debridement, resection of the three fistulas, removal of the femoral and acetabular componentswith the use of an osteotome,and irrigation using the pulse lavage system with physiological sodium chloride solution wereperformed.Due to the severestructural changes and a large medial wall defect in the proximal femur withimpairment of a large soft tissue zone, it was decided during operation not to put any other implants in the hip joint. All cultures from soft tissue samples collected from the hip joint region grew Staphylococcus aureus (resistant only to trimethoprim-sulfamethoxazole). The patient received antibiotic therapy with oxacillin for 16 days. On the 1stpost-operative day, the patient started physiotherapy– learned to sit in the bed and began passive exercises of the left hip joint. On the 2ndday,the patient began to stand and gradually learned to walk with crutch assistance. He was allowed touch-down weight-bearinguntil full wound healing. A soft left hip orthosis was applied to maintain muscle tone and decreaseedema. The surgical wound healed without any signs of infection and the skin clips were removed on the 14th post-operative day. The patient was discharged from the hospital and continuedantibiotic therapy with ciprofloxacin for 4 weeks. After 5 months, magnetic resonance imaging of the pelvic region showed fluid collections in the soft tissues of the left hip joint region (). The patient had an ultrasound-guidedleft hip joint fluid aspiration, but the acquired samples did not grow any microorganisms. After 1 year from the last surgery, the patient is relatively satisfied–he is walking with two crutches and can apply 10% of body weight on the left leg when standing. The patient has acceptable clinical results without antibiotic suppression therapy –edema in the left leg has decrease; there is no pain in the left hip region during movement and no active fistulas. C-reactive protein is 2.0mg/L.