Abstract: Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that has nodal and extranodal manifestations. Currently no guidelines are available for the management of this disease. We report a case of laryngeal RDD that did not present with classical symptoms such as fever or lymphadenopathy and could be easily misdiagnosed as malignant tumor. The patient received minimally invasive surgeries combined with steroid therapy to preserve the laryngeal function as much as possible, and a favorable clinical outcome was obtained. Reports of similar cases in literatures were reviewed to obtain a better understanding of the disease course, diagnosis and treatment of this uncommon condition.