Neurological symptoms can represent the first clinical manifestation of central nervous system (CNS) involvement in Hodgkin lymphoma (HL). Because of its rarity, it is often misunderstood for other pathological processes. We report two cases of pediatric CNS HL, presenting with neurological symptoms at diagnosis. We have also reviewed the literature and few cases are reported, only 19 of them concerning children. In both primary and metastatic CNS HL, all patients complained of neurological symptoms at presentation. Despite it being uncommon, physicians should regard the possibility of CNS localization in all children affected by HL presenting with neurological signs and/or symptoms.