Granular cell astrocytomas are uncommon tumors of the central nervous system (CNS) of which no cases have been documented in the spinal cord. This variant of glioma should not be confused with benign granular cell tumor which, although rare, has been well characterized in the spinal cord. We describe here the clinical, pathological, and radiological features of such an astrocytoma arising within the spinal cord at the dorsal level. A 48-year-old female was seen after about 1 year of dorsal pain and gradual spastic paraparesis. Magnetic resonance imaging (MRI) studies showed a 2-cm contrast-enhanced mass in the spinal cord at T6-T7, which had the appearance of an astrocytoma. At surgery, the tumor was found to be infiltrating a posterior column with no dural attachment. It was debulked and dissected. The histological diagnosis was astrocytoma with granular cell differentiation. In addition to documenting a unique example of intramedullary granular cell astrocytoma, we review the literature to investigate differences from other tumors with granular changes described in the spinal cord.