Behçet's disease is a multi-systemic vasculitis of small arteriolar and venular vessels, which shows a wide range of clinical manifestations, such as oral and genital aphthosis, erythema nodosum, panuveitis, complex gastrointestinal disorders, the early onset of neurological involvement being a negative prognostic factor in evolution. We present the case of a 36-year-old patient, who was admitted in the Clinic of Rheumatology for recurrent-neglected oral aphthosis, recurrent erythema nodosum, left hip pain, reduction of visual acuity of the right eye, weight loss, profuse sweating, marked fatigability. From the personal history was retained avascular necrosis of right femoral head, with arthroplasty at this level, human leukocyte antigen-B51 (HLA-B51) positive. Ophthalmological evaluation reveals severe bilateral optic neuropathy, with confirmation of neuro-Behçet's disease (NBD) diagnosis, in a Neuro-Ophthalmological Center, based on cerebral nuclear magnetic resonance and cerebrospinal fluid analysis. Associated corticosteroid therapy with Azathioprine was initiated, with no signs of activity and progression of the disease in evolution. The case provides a necessary upgrade of the therapeutic strategies specific to the NBD pattern, emphasizing the importance of the multidisciplinary approach of a patient with complex pathology.