Follicular lymphoma (FL), a common lymphoma in adults, occurs rarely in pediatric and young adult patients. Most pediatric cases have been described as Grade 3, but the criteria to distinguish the pediatric variant of FL (PFL) from usual FL (UFL) in adults are not well defined. We undertook a study of FL in patients under age 30. We identified 63 cases, which were analyzed by morphology, immunohistochemistry, and PCR analysis of IGH@ and IGK@ clonality. These data were correlated with clinical findings including stage, treatment, and outcome. Among the 63 cases, 34 cases were classified as PFL; 22 presenting in lymph nodes, 8 in Waldeyer’s ring and 4 in testis. Clonal immunoglobulin gene rearrangement was detected in 97% of PFL cases, but FISH analysis showed an absence of the BCL2/IGH@ translocation in all cases tested. Twenty-nine cases were classified as UFL, 28 of which presented in lymph nodes. The nodal PFL cases were observed exclusively in males in both children and young adults with a median age of 15 years. They showed marked head/neck predilection, blastoid cytological features with a high proliferation rate, lack of BCL2 protein and t(14;18), low clinical stage at presentation, and good prognosis. PFL involving Waldeyer’s ring were distinguished by MUM1 expression, 50% (3/6) of which carried IRF4 breaks. BCL2 expression was common (63%) in the absence of BCL2/IGH@ translocation. UFL cases were more common in females, exclusively in young adult patients (median age, 24 years) with no patients under age 18. 25/29 were grade 1–2, and four cases were classified as grade 3A. They exhibited a higher clinical stage at presentation. 83% expressed BCL2. Our results indicate that histological and immunophenotypic criteria can reliably separate PFL and UFL, and that UFL is exceptionally rare in the pediatric age group. PFL associated with particular anatomic sites have distinctive features, and should be evaluated separately in future clinical and biological studies.