Genodermatoses consign to an inherited skin disorder associated with structure andfunction. Several genodermatoses present with multisystem involvement lead to increased morbidityand mortality. Many of these disorders are rare and also have oral manifestations, called oralgenodermatoses. This article provides a focused review of molecular basis of importantgenodermatoses that affects the oral cavity and also has prominent associated dermatologic features.In several conditions discussed here, the oral findings are distinct and may provide the first clue of anunderlying genetic diagnosis. Many of these disorders are rare. However, the recognition of their skinfindings is important not only for the initiation of appropriate dermatologic therapy, but also for thedetection of other associated abnormalities in these frequently multisystem disorders, includingmalignancy.