Rosai-Dorfman disease (RDD) was formerly known as “sinus histiocytosis with massive lymphadenopathy”, and cases involving the gastrointestinal tract are rare. We present a case of pure extranodal RDD, resected as a polypoid lesion in colonoscopic study. The patient was a 62-year old woman with a history of sigmoidectomy for unexplained peritonitis. Microscopic study of the polypoid lesion showed the submucosal mass with histological and immunological features of RDD. The whole body computed tomography revealed neither lymphadenopathy nor tumor-like mass.
