Epidermolysis bullosa (EB) represents a spectrum of conditions that are characterized by blistering and mechanical fragility of the skin. There is genetic heterogeneity and marked variation in clinical phenotypes in the multiple EB disorders. The most recent classification recognizes four major EB grouping and over 30 EB subtypes. The severity of EB ranges from mild to severe skin involvement, and it can be localized or generalized. Oral features include repeated occurrence of blisters, erosions, and scars, which lead to limited mouth opening, ankyloglossia, elimination of buccal vestibule, and increased risk of oral carcinoma. Routine dental care or even normal tooth brushing might cause bullae on the oral mucosa. Occasionally, the clinician will be called upon to treat patients with EB and should therefore be aware of specific treatment modifications. we present a reviews of the literature with a case providing adequate anesthetic and surgical care.