AIM:To report unusual presentation and good outcome of neuromyelitis optica(NMO) in a child.METHODS:Case Report.RESULTS: An 8-year-old girl presented with 5-day history of sudden bilateral visual deterioration followed by left lower limb weakness. Visual acuity was perception to light in both eyes. Funduscopy revealed bilateral hyperaemic swollen optic discs. MRI of brain and spine revealed enhancing white matter lesions in the right frontal lobe and spinal cord at C5 level. She was diagnosed NMO and treated with intravenous methylprednisolone and tapering doses of oral prednisolone as maintenance therapy.Symptoms gradually improved 1 month after treatment. However, she presented with similar presentation 1 week after stopping oral prednisolone. MRI of brain and spine were reviewed and showed enhancing lesion in the right frontal lobe and longitudinal segment of spinal cord from C3 to C5 level. She was promptly given intravenous methylprednisolone for 5 days followed by prolonged tapering of oral prednisolone over 6 months period. After 2 months, she was able to walk and attend activities of school. Visual acuity was improved to 6/10 in both eyes and neurological examination was normal. There was no recurrence during the next year. Final visual acuity was 6/7.5 in the the right eye and 6/10 in the left eye.CONCLUSION: The diagnosis of NMO should be kept in mind although it is unusual presentation in child presented with bilateral visual loss and unilateral lower limb weakness. Early diagnosis and treatments would yield good outcome to the patient.