Neuromyelitis optica is a rare autoimmune demyelinating disease of the central nervous system in childhood. Its relapsing form is usually reported in adults. We report a 3-year-old girl with relapsing, IgG seropositive neuromyelitis optica. Initially she presented with optic neuritis, followed by three relapses with deterioration of optic neuritis and developing transverse myelitis. With each relapse, the treatment was less effective. Four years after the onset of the disease, the patient was blind, had paraplegia associated with urinary and bowel incontinence and short stature.