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Introduction: The authors report three cases of "Hyalinosis cutis et mucosae"; the first and the second case concern two siblings children (brother and sister) while the third is reported on a 42-year-old woman., Presentation of Cases: The case history and the personal anamnesis, the morphology of the skin lesions, the symptoms indicating laryngeal mucous affection as well as the histopathological picture, are compatible with the "Urbach-Wiethe disease". Furthermore, in the third case, the electron microscopic findings confirm the diagnosis., Discussion: The particularities of the present cases are discussed in connection with the corresponding bibliographical data.