Neurological signs and symptoms are commonly associated with both Hodgkin’s and non-Hodgkin’s lymphoma and are mostly attributed to either direct lymphomatous involvement of the nervous system, either as a result of extension to the spinal cord or nerves, or due to infiltration by lymphoma cells and drug toxicity. Guillain-Barre syndrome and its variants have been reported very infrequently in the literature. We present the case of a 70-year-old male admitted to the hospital for evaluation of uncontrolled hypertension. Incidentally, he was noted to have a low platelet count and a leukoerythroblastic picture in the peripheral blood. Two days into admission, he developed bilateral symmetrical ascending paresis consistent with acute acquired demyelinating polyneuropathy, a common variant of Guillain-Barre syndrome. At about the same time he developed worsening cytopenia and was diagnosed with diffuse large B cell lymphoma according to a bone marrow biopsy. The patient was treated with intravenous immunoglobulin for Guillain-Barre syndrome with significant improvement in muscle strength and subsequently treated with chemotherapy for his lymphoma.