Rosai-Dorfman disease (RDD) is a rare, acquired disease of unknown etiology that affects primarily children and young adults. It is characterized by a proliferation of distinctive histiocytes in the lymph nodes and/or extranodal sites. Involvement of the gastrointestinal tract is rare. We report a case of RDD in a 60-year-old woman who presented with hematochezia and was found to have RDD of the rectum presenting as a rectal mass. This report highlights the current pathogenetic mechanisms, immunohistochemical markers, and the gastrointestinal manifestations of RDD.
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