Since the advent of magnetic resonance imaging (MRI), to visualize lesions of multiple sclerosis has become easy to do. However, in some cases with primary spinal cord multiple sclerosis, it is not always easy to obtain a diagnosis in the first instance. We reported a case of primary spinal multiple sclerosis diagnosed through histological examination of a surgical specimen taken by an open biopsy. A 35-year-old woman was admitted with complaints of two-months duration of progressive weakness and sensory disturbance in the legs and buttocks. On radiological examinations including metrizamide CT myelography and MRI, enlargement of the conus medullaris was the only positive finding. Respective to her clinical course, intramedullary spinal cord tumor could not be ruled out, so an open biopsy was performed. Histological examination revealed that the cord lesion was acute demyelination with perivascular inflammation. Her neurological signs were almost completely cured with administration of corticosteroid, though new brainstem signs took place two months later and then a concrete diagnosis of her having multiple sclerosis was finally achieved. Since preoperative examinations can not differentiate spinal cord tumor from any other intramedullary cord lesions such as demyelinating foci of multiple sclerosis, surgical intervention would be approved in such atypical primary spinal cord multiple sclerosis.