BackgroundKindler's syndrome (KS) is a rare genodermatosis caused by a mutation in the KIND1 gene and characterized by skin fragility, photosensitivity, blistering, multi‐system involvement, and a risk of oral malignancy.Case PresentationTwo cases of KS in siblings are presented with severe cutaneous lesions, atrophic oral mucositis, chronic desquamative gingivitis‐periodontitis, candidiasis, and the novel finding of an imapacted mandibular distomolar. Topical steroids, antifungals, antiseptics, and oral prophylaxis caused marked improvement at the 2‐week follow‐up.ConclusionThe oral clinician plays a very significant role in a multi‐specialty team for diagnosis, management, surveillance for oral malignant transformation, and improving oral health‐related quality of life.