The celiac disease is an immunologic disorder, related to dietary gluten and morphologically characterized by a striking loss of villi in the small intestine and, with it, a marked reduction in the absorptive surface area. The authors experienced a rare case of pathologically confirmed malinant histiocytic lymphoma of jejunum, associated with celiac disease which was histologically manifested with ulcerative jejunitis in a 25-year-old Korean female who had suffered from projectile vomiting for 2 months. We report this case with literature review emphasis on the pathogenesis of malignant neoplasm in celiac disease and pathogenetic relationship between ulcerative jejunitis and celiac disease. To our knowledge, this is the first reported case of malignant histiocytic lymphoma complicating celiac disease with ulcerative jejunitis, in Korea.