Limb salvage surgery with wide margins is the gold standard of surgical treatment of sarcoma. Resection of nerves or vessels involved in the tumor is mandatory; however, resection of the bone in contact with the tumor is debated. We present a case of a malignant peripheral nerve sheath tumor in the distal thigh adherent to the femoral cortex. Due to femur involvement and histology malignancy, we planned to remove the involved bone. The patient underwent soft tissue sarcoma excision contextually with the distal femur resection and reconstruction with tumor prosthesis. The histological examination documented that the tumor had invaded the bone cortex. At the last follow-up, 3 years after surgery, the patient was continuously free of disease, the prosthesis had no complication, and the range of motion of the right knee was excellent. In conclusion, in our experience, the more aggressive the tumor is, the more radical the surgery must be, even including major surgeries, since removing only the periosteum is not enough to guarantee wide margins.