The Authors report the case of a 28-year-old patient with type 1 neurofibromatosis, who presented a pathological dislocation of the hip. This event was preceded by the development of a neurological pattern of spastic paraparesis and decreased pain sensation in both lower limbs, secondary to the progression of a dystrophic kyphoscoliosis. Pathological dislocation of the hip in neurofibromatosis has been reported in the literature as a consequence of intra-articular growth of neurofibromas. Conversely, in the case described here, clinical and radiographic features and the absence of neurofibromas in the joint, verified by means of radiographic and histological examinations, suggested the diagnosis of neuropathic arthropathy. The treatment of hip dislocation should be differentiated according to the aetiology, given the underlying articular instability in cases of neuropathic arthropathy.