Background: Kindler poikiloderma is an inherited autosomal genodermatosis characterized by blistering of the epidermis and mucosae. Its prevalence is unknown. Case report: We monitored two brothers suffering from this pathology. Oral manifestations mainly take the form of periodontal lesions. In our patients we noted gingivitis progressing to periodontitis as follow-up care was not effective. We also diagnosed enamel hypoplasia, described more rarely in this pathology. Conclusion: Periodontitis in Kindler Syndrome responds to maintenance therapy, but the absence of surveillance is penalized by a deterioration in periodontal condition and complication of management. All restorative, endodontic, surgical, periodontal and orthodontic treatments should be performed with appropriate precautions.