58-year-old woman with a history of xerophthalmia and xerostomia, asthenia and alopecia beginning at the age of 27, oral ulcers and recurrent episodes of bilateral parotitis. She attends rheumatology at the age of 52 (year 2016), without treatment. Laboratorial, ophthalmological and oral cavity examinations were performed, along with the biopsy of 2 minor salivary glands. Sjogren's syndrome (SS) was concluded, excluding other differential diagnosis. Hydroxychloroquine 400 mg/day was indicated, improving symptoms. After three years (year 2019), she added polyarthritis, large and recurrent oral ulcers and the appearance of genital ulcers. Behcet's syndrome (BS) was suspected and a genetic study was performed: HLA-B27 histocompatibility antigen negative, HLA Class I, locus B molecular typing: HLA B* 35 and HLA B*51 positive (nomenclature B35; B51 positive). This case is reported due to the infrequent association of SS and BS, both fulfilling the diagnostic criteria. A literature review was performed. Both pathologies were scarcelyn reported together in the literature. A common pathophysiological mechanism cannot be defined at the moment. It is intended to highlight the broad thinking of differential diagnoses in everyday situations in rheumatology. A high index of suspicion, a profuse history and correct physical examination are essential to arrive at the diagnosis.