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BACKGROUND Approximately 2%-10% of all central nervous system tumors are primary spinal cord tumors (SCTs). Spinal cord glioblastoma is a rare tumor type accounting for 1%-3% of all SCTs and 7.5% of all spinal cord gliomas. Notably, the small-cell variant of spinal cord glioblastoma is even rarer with only 2 previously reported cases. CASE DESCRIPTION We present herein a case report of a rare primary spinal cord glioblastoma in a 48-year-old patient with a 2-month history of numbness in the left arm and mild cervical pain radiating to the occipital zone. Clinical examination revealed hypoalgesia and thermal dissociation of the left arm and the ipsilateral superior part of the trunk treated through subtotal surgical resection followed by adjuvant chemotherapy and radiotherapy. Histologic examination of the surgical tumor specimen revealed features of the small-cell spinal cord glioblastoma. CONCLUSIONS To the best of our knowledge, this is only the third reported case of small-cell spinal cord glioblastoma. The aggressive nature of this tumor variant reduces overall survival rate regardless of the treatment.