We describe the case of a 59-year-old woman who had suffered from a typical Urbach-Wiethe disease since childhood and who complains of progressive mouth and ocular dryness since the age of 54 years. Xerostomia was severe with very poor salivation and was associated with a slight keratoconjunctivitis sicca. A specific lipoid proteinosis was evidenced in salivary glands (lower lip biopsy) with a PAS-positive hyalin-like deposit around vessels, capillaries and salivary gland canaliculi. An ultrastructural study showed the typical aspect of multilamination of the basal lamina of capillary vessels. Dysphonia and hoarseness are commonly found in Urbach-Wiethe disease, but, to our knowledge, a specific xerostomia has never been reported.