Intramedullary spinal cord metastasis (ISCM) was diagnosed in three dogs with signs of myelopathy. The clinicopathologic features of ISCM in these and previously reported cases in the veterinary and human literature were compared. Myelopathic signs associated with ISCM may be the initial clinical manifestation of malignancy or may develop in the patient with known malignancy. Pain, a frequent manifestation of extradural compressive myelopathy, is not a consistent feature of ISCM. Survey spinal radiographs are usually unrewarding and cerebrospinal fluid (CSF) abnormalities nonspecific. Myelography is indicated to differentiate intramedullary lesions from more common extradural compressive lesions. Myelographic interpretation may be difficult, and intramedullary tumors must be differentiated from spinal cord edema or hemorrhage. Evidence of widely disseminated malignancy should increase suspicion for ISCM; hemangiosarcoma and lymphosarcoma should be considered the most likely histologic types. CSF cytology may be helpful in the diagnosis of patients with lymphosarcoma. Prognosis is poor due to the frequent presence of disseminated disease, although temporary response to corticosteroid therapy may be achieved. More aggressive therapeutic approaches, such as spinal irradiation and microsurgical resection of metastases, have been advocated in humans but have not been reported in the dog. Although it is an uncommon complication of systemic malignancy, ISCM should be considered in the differential diagnosis of myelopathy in the dog.