<p>Epidermolysis bullosa is a rare skin disease. It belongs to the group of blistering genodermatoses and has substantial implications for dental care. Particularly in the dystrophic subtype, the condition commonly affects the skin and mucous membranes, which react to shear forces by forming blisters that heal with scarring. In addition to pain, frequent ulcerations, scarring, and later reduced tongue mobility and mouth opening render oral hygiene difficult. The caries risk is greatly elevated, and gingivitis and even periodontitis are often observed in children with epidermolysis bullosa. A sometimes pronounced lack of space presents dental professionals with major therapeutic challenges. The paradigm of tooth preservation is challenged in view of the poor prognosis for permanent molars. Nevertheless, with a suitable treatment concept, the oral health-related quality of life of those affected can be greatly improved.</p>