A 64-year-old gentleman was evaluated for right brachial weakness and recent memory disturbance on a background of an eight-month history of aching pain over the right upper limb. He developed progressive weakness in holding objects when a provisional diagnosis of right wrist drop was made elsewhere. MRI of the cervical spines showed mild degenerative changes. MRI of brain, vasculitic profile and EEG were unremarkable. There were no other significant symptoms or past medical history of concern. Systemic examination was within normal limits. Neurologically he was alert and well oriented. Recent and immediate memory disturbances were prominent with preserved remote memory. Language functions, speech, eye movements, and optic fundi were normal. Motor examination revealed a hypotonic and wasted right upper limb with early flexion contractures of the digits. Weakness and wasting were noted markedly over thenar eminence, interossei, tricep and supraspinatus. There was also wrist drop with marked hypotonia of wrist. Sensory examination revealed hypoaesthesia over the lateral arm, medial forearm, forearm extending to thumb and volar aspect of the mid forearm. Deep tendon reflexes over the right upper limb revealed diminished biceps and supinator jerks and absent triceps reflex. Electroneuromyographic studies revealed features suggestive of right brachial plexopathy. MRI of the brachial plexus revealed a lesion measuring 14. 7 x 0.37 cms, extending from the lower part of the brachial plexus, inferiorly the along the neurovascular bundle enhancing the axillary artery up to the upper third of the humerus. Intravenous methyl prednisolone (1 G/day for five days) was administered followed by significant pain relief and improvement in weakness. Open biopsy of the lesion revealed features suggestive of neurolymphomatosis. A diagnosis of primary peripheral neurolymphomatosis involving the brachial plexus was made. Chemotherapeutic regimen was instituted but patient was unfortunately lost to follow up after 3 months.