Langerhans cell histiocytosis (LCH) is a rare disease that is characterized by clonal neoplastic proliferation of the dendritic Langerhans cells. LCH presents either with solitary or multiple organ involvement.1,2 The most commonly affected organs include the bones, skin, and pituitary gland; the hematopoietic system, lymph nodes, and lungs are rarely affected.3 Tonsillar involvement is extremely rare. We report the case of a 36-year-old female patient with unusual unilateral tonsillar palatine involvement of LCH