Objective: Lipoid proteinosis, or Urbach-Wiethe disease, was first described in 1929. It is a rare recessive genetic disorder that causes a buildup of hyaline material in the skin and mucosa. The objective of this paper is to report a case of lipoid proteinosis, or Urbach Wiethe disease, emphasizing its otolaryngological manifestations. Method: A 38-year-old patient presented since young childhood with skin lesions on buttocks, face, arms, and legs and a weak cry. Currently, the patient has hoarseness that keeps her from talking on the telephone, anodontia, pale and stiff oral mucosa (specially frenulum and tongue), and narrowed nasal vestibules. Results: The clinical investigation included a videolaryngoscopy that showed an infiltration of the arytenoids and epiglottis. The vocal cords had normal mobility and coaptation. In order to confirm the diagnosis, a skin biopsy was performed, and the deposition of hyaline positive PAS material was found in the papillar derme. This rare disease does not have a specific treatment. The patient was treated with topical and systemic corticosteroids and also phonotherapy, obtaining some improvement. She was not interested in any surgical option. She is in clinical follow-up in Gaffrée Guinle University Hospital. Conclusion: Lipoid proteinosis is a rare disease, with approximately 300 reported cases, that has a chronic and benign course, still without a specific treatment. Because of the variety of symptoms in the oral cavity, larynx, and nose, it is important that the otolaryngologist is familiar with that affection.