Gastrointestinal stromal tumors (GISTs) are a rare tumor of the gastrointestinal tract and its primary origin from rectum is even rare^(1-3) We presented a case of GIST of rectum arising in a 61-year-old woman, in who the tumor was identified and the diagnosis was confirmed after abdominoperineal resection. Clinical and pathological features of the tumor and types of treatment were then discussed.