Congenital bullous pemphigus, because of successive episodes of erythema, serous or hematic vesicle formation and desquamation over the whole body including the oral cavity, causes severe problems for odontologists and anesthesiologists. The patient can only open his mouth partially and the fragility of the mucosa (development of bullae at the slightest touch of a dentist's instrument even if lubricated, during digital examination, etc.) makes intubation as well as surgical procedures very delicate propositions. The disease makes suturing of the oral mucosa impossible. These patients should receive regular dental care to avoid development of an advanced infection, knowing that the limitation of mouth opening only develops gradually as the disease progresses. Dermatologists should plan on monitoring and early routine care of teeth in any child with congenital bullous pemphigus.