A 50-year-old male presented with symptoms of a progressive spinal cord lesion over two and a half months investigations, including myelography, CT scan and magnetic resonance imaging (MRI), did not show cord compression although the MRI scan demonstrated thickening of the posterior cervical meninges. CSF examination revealed pleocytosis, increased total protein and cells suggestive of a lymphoproliferative disorder. Open lung biopsy of an asymptomatic left midzone mass was diagnostic of lymphomatoid granulomatosis. We believe this to be a case of lymphomatoid granulomatosis presenting as a progressive cervical myelopathy.