Ehlers-Danlos Syndrome (EDS) is one of the most common congenital connective tissue disorders, affecting the synthesis and production of collagen cells. The medical implications for joints, muscles, soft tissue, and blood circulation have been documented in the literature. Many oral manifestations are displayed in EDS patients, including gingival recession, lack of attached gingiva, early severe periodontitis, and dental anomalies and dental caries. However, the literature is limited and oftentimes contradictory, and therefore provides no consensus for guidelines regarding prosthodontic, periodontal, and implant treatment of patients with EDS. Therefore, the aim of this systematic review was to analyze the pertinent literature regarding prosthodontics, periodontology, and implant dentistry in EDS patients.