The author reports a case of Wiethe-Urbach syndrome (dermal and mucosal hyalinosis). The diagnosis was based on characteristic clinical findings in the skin and mucosal membranes and microscopic examination of the skin. Coexistent central nervous system dysplasia was characterized by brain atrophy, especially atrophy of the subcortical layers and two horn-like calcifications on the floor of the middle cranial fossa derived, most likely, from the dura.