Neurofibromatosis is an inherited disorder that causes skin discoloration and tumors. The musculoskeletal symptoms are specific, including bone deformities, dysplasia, joint instability, and osteoporosis. We present a rare case of a young patient with neurofibromatosis and multidirectional knee instability who underwent a successful complex primary knee replacement surgery. Stress right knee radiographs showed global joint instability with permanent anterior knee dislocation, excessively hypoplastic femoral condyles and patella, joint surfaces incongruency, and hypoplastic varus tibia, with intraluminal midshaft bone bridge causing severe stenosis. The patient could not walk, had an unstable recurvatum right knee, and used a wheelchair for her professional activities. The surgery involved a fully cemented rotating-hinged total knee arthroplasty with tibial and femoral stems. After three years of follow-up, the patient remains pain-free, fully ambulatory with no walking aids, a stable knee, a full range of motion, and no signs of aseptic loosening. This case highlights the decision-making difficulties and the significant surgical challenges faced during the operation.