Lymphomas are a broad category of cancers in which malignant cells form in the lymphatic system and they are among the most common cancers in the United States. Primary Central Nervous System Lymphoma (PCNSL) is a lymphoma affecting the structures which make up the central nervous system: the brain, spinal cord and meninges, with the pathology of most cases belonging to the diffuse large B-cell category. Neurocognitive symptoms of PCNSL range in severity and onset, depending on the location of the tumor and the health of the patient. The prognosis of PCNSL upon diagnosis is poor, with 5-year survival rates of around 33%, contingent on patient age, gender, race, initial health, presence of autoimmune disorders or other comorbidities and access to treatments. There is a clear role of Human Immunodeficiency Virus and Epstein-Barr Virus in lymphomagenesis, making these two diseases a major risk factor for the development of PCNSL. The treatments for PCNSL are most commonly high-dosage chemotherapy and whole brain radiation, with some cases requiring surgery. The exploration of the efficacy of autologous stem cell transplants in combination with high-dosage chemotherapy is an emerging area of study with great promise for the extension of overall survival rates and quality of life of PCNSL patients.