Follicular lymphoma, although common in adults, is rare in children. Pediatric follicular lymphoma has a more favorable prognosis than adult follicular lymphoma, even though it is often of higher grade. Children with follicular lymphomas are generally at a lower clinical stage, respond well to less aggressive therapy, and have a better survival than adults. Follicular lymphoma must be distinguished from reactive follicular hyperplasia, which it may mimic. Immunohistochemical and molecular markers serve to facilitate this distinction, as well as careful attention to clinical and morphologic details. It is important to recognize pediatric follicular lymphoma as a unique clinicopathologic entity to properly diagnose and manage these patients. It may represent a subset of follicular lymphoma with a particularly good prognosis.