Cerebral amyloid angiopathy (CAA) is a central nervous system disorder characterized by the deposition of amyloid-β peptides within vascular walls, resulting in disruption of normal neural structures. Although CAA may remain asymptomatic, it can also present with a wide spectrum of clinical manifestations, ranging from headache to severe complications such as intracranial hemorrhage or dementia. We report a patient with an intramedullary lesion at the C6-T1 level of the spinal cord that mimicked an intramedullary tumor or inflammatory lesion and presented with arm weakness and pain. An open surgical biopsy followed by histological examination confirmed the diagnosis of spinal cord amyloid angiopathy (SCAA). Postoperative evaluation revealed no evidence of systemic amyloidosis or a familial form of the disease. We describe an exceedingly rare case of SCAA. Solitary SCAA should be considered in the differential diagnosis of progressive intramedullary lesions that resemble spinal cord tumors.