Vascular Ehlers-Danlos syndrome (vEDS) is a rare connective tissue disorder with high risk of spontaneous bowel perforation. The association between vEDS and inflammatory bowel disease is not clearly established. This article reviews isolated cases described in the literature associated or overlapping inflammatory bowel diseases (IBD) with EDS. CAse-BAsed REview sTandards were use and a case description of vEDS with confirmed bowel inflammation was presented as a basis of this analysis. None of the articles found were directly related to the vEDS, therefore the case presented uniquely demonstrates the problem as well as showed successful use of biological therapy of patient with vEDS. This suggests an inflammatory component in vEDS pathophysiology. The potential for bowel intestinal inflammation to occur in a condition previously considered non- inflammatory offers a valuable basis for further discussion. The article highlights the need to consider inflammation and select appropriate diagnostic methods, while also paving the way for treating the inflammatory process including biologics in vEDS.