Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonneoplastic histiocytic disorder that often involves the lymph nodes and occasionally the central nervous system or skeleton. However, the presence of extranodal Rosai-Dorfman lesions without lymphadenopathy is extremely rare. Here we present a case of a pathological confirmed Rosai-Dorfman disease with pituitary and skeletal involvement without lymph nodes involvement.