Epidermolysis bullosa dystrophica is rare, chronic non-inflammatory disease of hereditary trait, which easily ptoduces bullae spontaneously or by heat and minor trauma such as pressure or friction. We present two cases of epidermolysis bullosa dystrophica that seems to be a dominant type considering the presence of family history with histopathologic and electron microscopic findings. Successful anesthetic management of two patients with epidermolysis bullosa dystrophica was accomplished with general anesthesia.. The special concerns which relate to airway management in this disease are discussed. Early diagnosis and institution of preventive measures can minimize the need for restorative and surgical management in these children. However, when anesthetic management is necessary, the use of appropriate consultants and adjuncts can provide valuable support.