Lipoid proteinosis (LP) is an uncommon, autosomal, recessively inherited disorder. It is typically characterized by hoarseness from early infancy, together with various cutaneous manifestations such as waxy papules, acneiform scarring, and eyelid beading. A 42-year-old woman was admitted to our dermatology outpatient clinic complaining of rigidity of the oral mucosa and limitation in tongue movement. She also had a burning sensation and decreased taste perception in the mouth while eating, as well as hoarseness of voice. She was diagnosed with lipoid proteinosis. The typical cutaneous manifestations of LP include waxy yellow papules with generalized skin thickening on the face, extremities, and trunk. Here we report a case with significant mucosal involvement but less skin involvement.