Primary spinal melanoma is a very rare condition: to date < 60 cases have been reported in the literature. A 48-year-old man presented with a 6-month history of upper- and lower-extremity numbness. Spinal magnetic resonance imaging (MRI) revealed a space-occupying lesion at the C2-C6 level. This was confirmed as a melanoma by immunohistochemistry. Cerebral MRI showed multiple lesions with the same signal characteristics as those seen in the spinal lesion on MRI. Complete skin, mucosal and retinal examination failed to show any primary lesion, therefore a diagnosis of primary cervical melanoma with brain metastases was made. To our knowledge this is the first report of a primary melanoma of the cervical spine with cerebral metastases at the time of diagnosis. This article presents pertinent reported literature and discusses the aetiology, diagnosis, treatment and prognosis of this unusual condition.