Primary gastrointestinal follicular lymphoma (FL) is rare, and there is only one previously reported case with lymphoma cells confined within the mucosa, a stage IE disease. We present the second case of primary intramucosal FL located in the terminal ileum, with focal nodal involvement in a 47-year-old male. This unusual case demonstrates that even an intramucosal FL exhibits the potential for nodal spread, which is evidence of lymphocytic trafficking within the lymphoid system.