Intravascular lymphoma (IVL) is a rare subtype of extranodal diffuse large B-cell lymphoma. It is characterized by proliferation of neoplastic Lymphoid cells almost exclusively within the lumina of small blood vessels. It can affect virtually every organ system. Due to its rarity and its diverse and heterogeneous clinical presentation, diagnosis is difficult and often made post-mortem. When diagnosed early, it is, however, potentially treatable. We present a young woman with longstanding constitutional symptoms, positive antinuclear antibody, elevated LDH levels and rapidly progressive encephalopathy. FDG-PET scan showed intense uptake in the renal cortex, which prompted us to perform a kidney biopsy which was compatible with IVL. The value of PET in establishing the diagnosis of this rare disease will be discussed.