Context: Behçet's disease is a rare chronic-recurrent rheumatic disease characterized by the presence of multisystemic vasculitis with mucocutaneous and ocular manifestations. The origin is unknown, although it is believed to appear in genetically predisposed individuals who are exposed to some external agent. Objective: With regard to one case, to perform a bibliographic review of Behçet's disease, in terms of prevalence, clinical manifestations, differential diagnosis, gynecological management and complications. Material and methods: A 42-year-old female patient, of South American origin, G3P3A0, with a history of recurrent oral ulcers and genitalia for 23 years. She went to gynecological urgencies due to ulcerative genital lesion in the left, painful, exudative major left upper lip of 4 cm. She had 3 painful ulcerated oral lesions and thrombophlebitis. No ocular lesions or other symptoms. Main Outcome/ Results: In the first gynecological examination, a 2 x 2 cm vulvar ulcerated lesion biopsy was performed, with anatomopathological results of acute and chronic inflammatory infiltrates of lymphocytes and PMN. ETS was ruled out. In the second consultation: an ulcerated lesion of 3 x 2 cm with raised and erythematous edges was observed. A sterile fibrinoid-like exudate is targeted. Corticosteroid therapy and antibiotics were prescribed, with improvement of the clinic. It also requested complete analytical and autoimmunity tests. Ac anti-DNA, ANCA, Ac myeloperoxidase and anti-proteinase 3, HLA-B51, HLA-B27, HLA B57: 01 that were negative. Conclusions: In view of a clinical picture of recurrent oral and genital ulcers, we must rule out a Behçet syndrome, since these are the main and most common clinical manifestation of the disease. There are criteria established by the International Behçet Disease Study Group for the diagnosis, prevention of complications and symptomatic treatment. The most effective management involves an early diagnosis and clinical intervention with continuous follow-up. As a consequence, it will be possible to reduce the risk of serious complications and socioeconomic costs due to Behçet's disease.