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Epidermolysis bullosa (EB) is a rare inherited group of genodermatoses characterized by mucocutaneous fragility and blister formation, either spontaneously or as a result of minimal mechanical trauma. The repetition of these episodes in the oral cavity leads to atrophy of the mucosa, causing microstomia, ankyloglossia, tongue denudation, and vestibule obliteration, characteristics that make dental treatment difficult. Patients with EB are at high risk for caries due to the presence of dental anomalies; they also tend to have a soft diet and difficulties with mechanical removal of the dental biofilm. This case report presents a patient diagnosed with EB and describes the difficulties faced by the clinician during dental treatment as well as the measures adopted to safely manage the patient's dental care.