Ehelr Danlos Syndrome (EDS) is characterized by hyperextensibility of skin, hypermobility of joints n resulting in dislocations, and tissue fragility. It is a clinically and genetically heterogeneous connective tissue disorder affecting 1 in 5,000 individuals. Heterogeneity of it makes accurate diagnosis difficult. Preventive and supportive treatment and regular monitoring of patient’s oral and physical status may be helpful in treating this kind of patient. Pedodontists have important role in initial diagnosis and preventive as well as corrective dental care of EDS patients.