Primary colorectal lymphoma is an infrequent disease of unknown origin and with a growing incidence. Primary colorectal lymphoma accounts for only about 0.2% of large intestinal malignancies. The aim of this study is to review, identify and underline risk factors, presentation, treatment and prognosis of primary colorectal lymphoma, using the three most important studies made in the last years: the Chung-Wei Fan study from the division of colon and rectal surgery, in Taipei [Fan CW, Changchien CR, Wang JY. Primary colorectal lymphoma. Disease of the Colon and Rectum 2000;43:1277-82]; the Wong and Eu [Primary colorectal lymphomas. Colorectal Diseases 2006;8:586-91] study from the Department of colorectal surgery in Singapore and the study of Doolabh et al. [Primary colon lymphoma. Journal of Surgical Oncology 2000;74:257-62] University of Texas Southwestern Medical School, Dallas, Texas.