Primary Central Nervous System Lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma that primarily affects the central nervous system, including the brain, spinal cord, and eyes. Unlike other types of lymphomas, PCNSL is confined to these specific areas and does not typically involve other parts of the body. This type of lymphoma is often seen in individuals with compromised immune systems, such as those with HIV/AIDS or individuals undergoing immunosuppressive therapy. However, it can also occur in immunocompetent individuals, albeit less commonly. The symptoms of PCNSL can vary but may include neurological deficits, headaches, cognitive changes, and visual disturbances. Due to the location of the tumor within the central nervous system, diagnosis often involves brain imaging, cerebrospinal fluid analysis, and, in some cases, a brain biopsy. Treatment for PCNSL typically involves a combination of high-dose chemotherapy and radiation therapy.The choice of treatment depends on factors such as the patient's overall health, the extent of the disease, and other individual considerations. Prognosis for PCNSL varies, and successful outcomes are often associated with early diagnosis and appropriate treatment. However, challenges exist due to the potential impact on neurological function and the limited penetration of some treatments through the blood-brain barrier. Ongoing research aims to better understand the biology of PCNSL and develop more targeted therapies to improve outcomes for affected individuals. As PCNSL is a complex and relatively uncommon condition, a multidisciplinary approach involving neurologists, oncologists, and other specialists is crucial for optimal management and care.