Purpose: Ehlers-Danlos syndromes are a group of inherited connective tissue disorders, with those with the hypermobile subtype (hEDS) potentially suffering from a range of gastrointestinal symptoms, such as abdominal pain and early satiety. It has been suggested that abdominal vascular compression syndromes (AVCS) such as median arcuate ligament syndrome, superior mesenteric artery syndrome, nutcracker syndrome, and May-Thurner syndrome, may be causative of these symptoms in hEDS. However, the diagnosis is challenging due to the lack of awareness and unclear diagnostic imaging criteria. Moreover, there is limited knowledge regarding open surgical repair for ACVS in hEDS. The purpose of this review was to critically appraise the current literature on the diagnosis and surgical management of AVCS in hEDS. Methodology: This was a scoping review of the literature. The study protocol followed PRISMA-ScR. A literature search of Ovid MEDLINE, EMBASE, and Google Scholar was performed using standardised search terms. Inclusion criteria were, 1) involved one more AVCS, 2) described diagnosis or surgical management, and 3) involved patients with hEDS. Screening and data charting was conducted in a standardised fashion. Results: Overall, 213 citations were screened, of which 20 were included. This represented 408 patients, of which 214 had hEDS. 15 studies described diagnosis and 19 reported interventions. Studies described a wide range of diagnostic imaging modalities with generally heterogenous criteria, significant subjectivity, and a lack of validation. Studies reporting surgical management of ACVS similar described a large range of treatment options, but there was no standardisation of reporting by objective measures, limited reporting of complications, and no long-term follow-up. Conclusion: There is a small volume of heterogenous literature regarding the diagnosis and surgical management of ACVS in hEDS. Robust data is needed before management guidelines can be developed.