Neurolymphomatosis (NL) is a rare neurological manifestation of lymphoma characterized by malignant lymphoma cells infiltrating cranial or peripheral nerve, or their roots. We present the first reported Australian case of a patient whose initial presentation of relapsed mantle cell lymphoma was NL. Our case highlights that clinical and imaging findings of NL often mimic other neuropathies, and hence presents unique challenges that may lead to delayed diagnosis and management. We emphasize the importance of considering NL in the differential diagnosis and combining imaging with other diagnostic modalities such as lumbar puncture (LP) to aid in the diagnosis of NL particularly where there is acute neurological deterioration.