Primary Central Nervous System Lymphoma (PCNSL) is a rare, aggressive extranodal non-Hodgkin lymphoma. It is critical to get a diagnosis and start therapy as soon as possible to improve clinical results. Despite a new medicinal strategy that has increased survivability, the survival rate is still quite low. This report presents a new case of PCNSL that appears in an immunocompetent patient with two different rare genetic rearrangements and a necrotic histological appearance.