Epidermolysis bullosa (EB) is a rare congenital disease characterized by the formation of blisters following minimal trauma. Oral health can be compromised by oral bullae, perioral fibrosis, ankyloglossia, and a high risk for dental caries, all of which lead to a decreased quality of life. The purpose of this paper is to report the case of an 11-year-old female who presented with autosomal recessive dystrophic EB. Besides the skin manifestations, the extra- and intra-oral exams revealed lesions on the lips and mucosa, microstomia, ankyloglossia, depapillated tongue, and carious teeth. This case emphasizes that patients diagnosed with EB require multidisciplinary care, with the dentist playing an important role in oral health management.