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Patient: Male, 43Final Diagnosis: NeurofibromatosisSymptoms: Hip painMedication: —Clinical Procedure: —Specialty: Orthopedics and TraumatologyObjective:Rare diseaseBackground:Neurofibromatosis type 1 is a disease known for orthopedic manifestations such as spine deformities, congenital pseudarthrosis of the tibia and other bony dysplasias; joint dislocations are rare. Joint arthritis caused by neurofibromatosis, with a stable hip, has never been reported in the English literature before.Case Report:A 43-year-old man diagnosed with neurofibromatosis type 1 (NF-1) presented with hip pain of 5 years duration, with no history of trauma and no clinical or radiological signs of dislocation. He had classical clinical and radiological signs of hip arthritis, with acetabular erosions and femoral head arthritis. The patient was treated with total hip arthroplasty metal-on-metal (MoM).Conclusions:Hip arthritis alone without subluxation or dislocation due to NF-1 is an extremely rare presentation. This is the first reported presentation and provisional treatment with total hip arthroplasty.