Behçet’s syndrome (BS) is an autoimmune, rare, and severe multisystemic inflammatory disease characterized by recurrent oral aphthous ulcers, genital ulcers, skin lesions, and both anterior and posterior uveitis; articular, vascular, gastroenteric and neurological involvement may also occur. The multi-organ involvement and the wide range of clinical spectrum make the diagnosis of BS challenging. As other systemic chronic diseases, BS can affect all aspects of patients’ life, including sexual dysfunction (SD). So far, SD has been deeply studied among patients affected by many rheumatic diseases; however, little is known about the prevalence and the characteristics of SD among BS patients. The present work was aimed at providing a systematic literature review of the literature published on SD and BS. We carried out a systematic search in PubMed and Scopus based on controlled terms (MeSH) and keywords to identify literature data on SD in BS. The systematic search was independently performed by two clinicians based on the controlled medical subject heading terms for Behcet syndrome and SD. Systematic database search identified 32 records. Screening by title and abstract was then conducted and a total of 10 articles were eligible for full text assessment, five studies explored SD in male patients with BS, 3 studies in females and 2 works reported data on both genders. Globally, the systematic literature review results have underlined that SD seems frequent in BS patients. Both female and male patients experienced a significant correlation between SD and depression. The studies investigating sexuality in BS seem to demonstrate that in these patients SD may be mainly related to depression rather than to active organic manifestations.