Xanthoma disseminatum (XD) is a benign, non-Langerhans cell histiocytosis characterized by disseminated xanthomatous lesions with face, flexures, and mucosa. Approximately 100 cases have been reported in the literature. Most of XD develops in mucocutaneous location including skin, oral cavity and pharynx, however laryngeal involvement is rare. While the natural course of xanthoma disseminatum is usually benign and often self-limiting, but XD develop in critical anatomical locations may result in morbidity and mortality. We report a 59-year-old man who presented with respiratory symptoms because of laryngeal involvement of XD. Laryngeal XD was removed by CO2 laser via transoral approach and the symptoms were relieved.