Acute disseminated encephalomyelitis (ADEM) is considered a monophasic acute demyelinating disorder of the central nervous system characterized by diffuse neurologic signs and symptoms coupled with evidence of multifocal lesions of demyelination on neuroimaging. The common presenting symptoms are lethargy, fever, vomiting, headache, meningeal signs, and seizures. Neurological manifestations included altered sensorium, multiple cranial nerve involvement, quadriplegia and paraplegia, dystonia and choreiform movements, ataxia, nystagmus, bladder involvement, speech defect, and double vision. Peripheral facial nerve palsy rarely accompanies. We describe the case of a 7-year-old girl who developed acute disseminated encephalomyelitis with right peripheral facial paralysis. She was admitted to the hospital with visual impairment, depressed mood, gait disturbance, and deterioration of facial expressions. She had horizontal and vertical nystagmus, right peripheral facial paralysis, ataxic gait, and hyperactive deep tendon reflexes. The cerebrospinal fluid (CSF) examination was normal. Cerebral magnetic resonance imaging (MRI) showed multiple white matter lesions. We observed a significant improvement in clinical findings with high dose methylprednisolone pulse therapy.