Rosai Dorfman disease is a rare non Langerhans histiocytic disease with intranodal and/or extranodal manifestations, most commonly affecting cervical lymph nodes. There is no treatment guideline for this disease. This paper reports a case of infraglottic portion, which did not show typical symptoms, such as fever or lymphadenopathy, and was easily misdiagnosed as malignant tumor preoperatively. The patient underwent low-temperature plasma minimally invasive surgery combined with hormone therapy and achieved good clinical effect. By reviewing the literature, we can better understand the clinical manifestations, diagnosis and treatment of this rare disease.