Follicular lymphoma of the gastrointestinal tract is a rare entity accounting for <
7% of all non-Hodgkin lymphomas and is more common in the small intestine, whereas colorectal manifestations account for only 1%-2% of the cases. Most patients have limited disease with promising overall survival in comparison to nodal lymphoma although the morphologic, immunophenotypic, and genetic aspects of nodal follicular lymphomas remain the same. Despite the lack of randomized clinical trials, chemotherapy is frequently used to treat NCCN grade III and IV colonic follicular lymphomas with favorable prognosis. We hereby present a case of follicular lymphoma of the colon that was treated conservatively with a successful outcome.