Lipomas are benign mesenchymal tumors that are often found in the head and neck. Intrinsic lipomas of the larynx and supraglottic area are rare, as fewer than 115 cases have been reported in the literature; almost all of these occurred in isolation. We report a case of a laryngeal lipoma that was associated with diffuse systemic lipomatosis. The tumor was successfully removed via an endoscopic laser resection. To the best of our knowledge, this is only the second case of laryngeal lipoma associated with lipomatosis to be reported in the English-language literature. We also review the literature on head and neck lipomas.