To determine the accuracy of magnetic resonance imaging for diagnosing cervical stenotic myelopathy in horses, 39 horses with spinal ataxia and 20 control horses underwent clinical and neurologic examinations, cervical radiographs, euthanasia, magnetic resonance (MR) imaging of the cervical spine and necropsy. Twenty-four horses were diagnosed with cervical stenotic myelopathy, 5 with cervical vertebral stenosis, 7 with idiopathic ataxia, 3 horses had other causes of ataxia, and 20 were controls. The MR images were assessed for spinal cord intensity changes, presence of spinal cord compression, spinal cord compression direction, shape of spinal cord, and the presence of synovial cysts, joint mice, and degenerative joint disease. The height, width, and area of the spinal cord, dural tube and vertebral canal were measured. The identification of spinal cord compression on MR images was significantly different in horses with cervical stenotic myelopathy (P < 0.02), but in the cervical stenotic myelopathy group the identification of spinal cord compression on MR images had poor to slight agreement with histopathologic evidence of compression (κ = 0.05). Horses with cervical stenotic myelopathy were more likely to have a T2 hyperintensity in the spinal cord (P < 0.05). Horses with cervical stenotic myelopathy or cervical vertebral stenosis were more likely to have degenerative joint disease than control horses or horses with other or idiopathic ataxia.