Chronic osteomyelitis caused by Staphylococcus aureus small colony variants in combination with osteopetrosis is a unique combination of disorders that confronted us with major challenges. The therapeutic approach included four serial debridements and antimicrobial therapy. The aggressive treatment led to an instability of the brittle and hard osteopetrotic bone, and after 11 weeks, a fracture of the femoral neck occurred. A salvage procedure of the femur was performed, and the cultures obtained during this intervention remained negative. At a 6-year follow-up, the girdlestone situation still showed an acceptable functional outcome without any recurrence of osteomyelitis.