A fever of unknown origin is often pursued diagnostically under the framework of infectious, rheumatologic, and neoplastic causes. When encephalopathy ensues, the differential diagnosis narrows, but can remain elusive, particularly when dealing with rare diseases. We present the case of a patient with fever of unknown origin and intermittent encephalopathy that spanned multiple hospital admissions and ultimately yielded a diagnosis of intravascular large B cell lymphoma complicated by hemophagocytic lymphohistiocytosis. We review the varying presentations of this disease, when to consider this as a diagnosis, and how to most accurately make the diagnosis.
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