Acute necrotizing encephalopathy of childhood is a rare acute encephalopathy occurring after febrile illness with subsequent rapid deterioration of consciousness and convulsions. Typical brain magnetic resonance imaging (MRI) findings are symmetric involvement of the bilateral thalami, brainstem, and cerebral periventricular white matter or the cerebellum. Spinal cord involvement has never been reported. This report describes a healthy 2-year-old girl, who displayed typical clinical manifestations of acute necrotizing encephalopathy. Brain MRI demonstrated symmetrical lesions over the bilateral thalami and periventricular white matter. The clinical features and radiologic images suggested spinal cord involvement. This is the first reported case of acute necrotizing encephalopathy with spinal cord involvement. Spinal cord lesions are, therefore, possible in cases of acute necrotizing encephalopathy of childhood, and these require special attention.