Occipital neuralgia has been attributed to lesions at a peripheral nerve or radicular level. On rare occasions, it has been associated with cervical cord lesions. We report a 55-year-old woman who presented with an isolated occipital neuralgia and was found on further investigation to have a restricted, isolated myelitis at C2 level. This represents the second reported case of occipital neuralgia due to C2 myelitis and should alert clinicians to considering cervical MRI in patients with occipital neuralgia.