Epidermolysis bullosa is a hereditary disease characterized by bullae in the skin and/or gastrointestinal tract. As a result of the physical and metabolic changes caused by this disease, various difficulties can arise in the practice of anesthesia. In the pediatric patient who was sedated for dental treatment, the difficulty in accessing the intravenous way and the difficulty of monitoring the patient. Despite all these difficulties, successful and uncomplicated management of the anesthesia was achieved in the patient.