Lymphoma, a cancer of lymphoid cells, classified as Hodgkin lymphoma (HL) and non-Hodgkin Lymphoma (NHL), is the third most common type of cancer in children, following acute leukemia and central nervous system (CNS) tumors, and is responsible for approximately 10–15% of all pediatric malignancies [1], [2]. In particular, the lymphoma incidence rate stands at 1.7 cases per 100,000 children [3]. While the pathogenesis of lymphomas is not fully understood, the development of lymphoma in children is believed to be multifactorial, with genetic, environmental, and infectious factors all playing a role. Over the past few decades, further investigations have been carried out to discover new frontiers in the pathogenesis of lymphoma, enabling the development of innovative therapeutic approaches and achieving more favorable treatment outcomes [1], [2]. As a result, significant progress has been made in the treatment of lymphoma, with high rates of remission and survival observed in many cases over the years [4], [5], [6]. Chemotherapy, radiation therapy, stem cell transplantation, and, less often, surgery are the most common treatments for lymphoma in children [1], [7]. Although these treatments have improved outcomes for patients with lymphoma, they are associated with significant short- and long-term toxicities, which shows the need for alternatives with lower toxicities [8]. The latest advancements in targeted therapies and precision medicine have resulted in the development of novel treatments, such as cancer immunotherapy approaches, which harness the power of the immune system to identify and combat cancer cells. These cutting-edge treatments have shown promising results in treating lymphomas [8], [9]. The emergence of immune checkpoint inhibitors (ICIs), as a novel therapeutic approach to cancer, has sparked hope for more effective and less toxic treatments for solid tumors and hematologic malignancies [9], [10]. ICIs target molecules called immune checkpoints, such as programmed cell death protein 1 (PD-1) and cytotoxic T lymphocyte-associated protein 4 (CTLA-4), which are involved in regulating T cell activation and immune tolerance. ICIs therapy in adult cancers offers the advantage of long-lasting remissions and a low rate of severe toxicities in clinical trials, however, its applicability is limited to specific types of cancer due to the nature of this therapy [11]. The FDA has approved several ICIs to treat a variety of adult cancers, but their potential to treat pediatric malignancies is still under investigation [12]. The use of ICIs in pediatric lymphoma is a relatively new field, and there is still much to learn about their effectiveness, safety, and optimal use [13], [14]. This review provides an overview of immune checkpoint inhibitors (ICIs) in pediatric lymphoma treatment, discussing their significance and potential role, summarizing results from clinical trials, and identifying areas for further research to advance knowledge on ICIs use in this population.