Background: Facial pain resembling trigeminal neuralgia is not a common clinical feature of cervical spinal cord tumor. Depending on nature of the facial pain, differential diagnosis tends to include neurovascular conflict, multiple sclerosis, cerebellopontine angle tumors, herpes zoster, facial injuries, and other conditions involving trigeminal nerve, ganglion, and root. Here, we present a unique case of pain in trigeminal distribution due to an intramedullary tumor in the upper cervical spinal cord. Case Description: A 27-year-old male was admitted with complaints of intense facial pain on the right side lasting for several years. MRI revealed an intramedullary lesion at the C1 level and no signs of a neurovascular conflict or a demyelination. This lesion was removed microsurgically, with the subtotal resection immediately abolishing the pain and causing no additional neurological deficit. Histological analysis revealed ganglioglioma, Grade 1. After 5-day hospital stay, the patient was discharged home; 2-year follow-up showed no tumor recurrence on MRI and persistent relief of facial pain. Conclusion: Secondary trigeminal neuralgia may be explained by a pathological process in vicinity of the spinal trigeminal nuclei. Removing the tumor may be expected to provide complete and lasting pain relief.