The neurological symptoms and the magnetic resonance imaging (MRI) findings in a patient with a spinal pencil-shaped form of softening were compared with the autopsy findings. The distribution and shape of the lesion on MRI, especially on T2-weighted images, correlated well with the autopsy findings at the level between the medulla oblongata and the T2 segment. But these findings did not necessarily correlate with the neurological abnormalities. A pencil-shaped lesion that had extended cephaladly, and which was seen on MRI and at autopsy, did not manifest neurological deficit. MRI is useful to aid the clinical diagnosis of spinal cord pencil-shaped softening.