Nervous system involvement affects up to one-third of patients with lymphoma, via a variety of mechanisms ranging from direct invasion, to demyelination, paraneoplastic processes, and treatment related complications. Nervous system involvement can manifest at any location and occur at any stage of lymphoma, often resulting in distinct and atypical patterns. Here we describe three patients, presenting each with motor predominant polyradiculoneuropathy and cranial neuropathy, transient encephalitis, and frontal gait dysfunction. Through further workup, all were diagnosed with lymphoma, with their neurological manifestations directly or indirectly related to the underlying lymphoma. Our study serves as an alert that in patients presenting with unusual neurological symptoms, lymphoma should be a consideration on the differential diagnosis.