Background: Ehlers-Danlos syndrome (EDS) is a hereditary disorder that affects the connective tissue and collagen structures in the body characterised by joint hypermobility, skin hyperextensibility and tissue fragility. Objective: The aim was to investigate temporomandibular disorders (TMD), bite force, teeth in occlusal contact and osseous changes of the temporomandibular joints (TMJs) in 26 patients with hypermobile EDS (hEDS), differentiated by a genetic test, compared to 39 healthy controls. Methods: Clinical examination according to Diagnostic Criteria for Temporomandibular Disorders (DC/TMD), radiological examinations of the TMJs by cone-beam-computed tomographic (CBCT) scans, registration of bite force and teeth in occlusal contact was performed. Statistical analyses included Fisher's Exact Test, multiple logistic and linear regression models adjusted for age, gender and Body Mass Index (BMI). Results: Single symptoms and signs of TMD occurred significantly more often in hEDS (p =.002; p =.001; p =.003; p = <.0001; p =.012) and maximum mouth opening was significantly smaller in hEDS compared to controls (p = <.0001). The DC/TMD diagnosis myalgia, myofascial pain with referral, arthralgia, headache attributed to TMD, disc displacement disorders and degenerative joint disease occurred significantly more often in hEDS compared to controls (p =.000; p =.008; p =.003; p =.000; p = <.0001; p =.010, respectively). No significant differences were found in bite force and in teeth in occlusal contact between the groups (p >.05). On CBCT of the TMJs, subcortical sclerosis occurred significantly more often in hEDS compared to controls (p =.005). Conclusion: Symptoms and signs of TMD and osseous changes of the TMJs occurred significantly more often in hEDS. Bite force and teeth in occlusal contact were comparable to controls.