Neuromyelitis optica (NMO) is a rare autoimmune inflammatory demyelinating disorder of the central nervous system, predominantly affecting the optic nerves and spinal cord. Pediatric cases are uncommon and often underdiagnosed due to heterogeneous clinical and radiological presentations. We report two pediatric cases of NMO with distinct clinical and immunological features. Case 1: A 10-year-old girl presented with a 5-day history of progressive bilateral visual loss and impaired color vision. Ophthalmological examination revealed severe bilateral visual acuity reduction and grade II papilledema. Brain and spinal MRI showed T2 hyperintensity of the optic nerves and chiasm with cervical spinal cord involvement (C2–C7). Cerebrospinal fluid analysis showed mild hyperproteinorrachia. Anti-MOG antibodies were positive. The patient was treated with intravenous methylprednisolone followed by oral corticosteroids and intravenous immunoglobulins, with significant clinical improvement within 8 days. Case 2: An 8-year-old girl presented with a preceding flu-like illness followed by acute paraplegia, sphincter dysfunction, and bilateral visual impairment. Neurological examination revealed severe motor and sensory deficits consistent with longitudinal extensive transverse myelitis. MRI demonstrated diffuse spinal cord involvement with gadolinium enhancement and normal brain imaging. Laboratory tests showed elevated inflammatory markers and positive antinuclear antibodies. She received high-dose intravenous corticosteroids followed by oral prednisone and azathioprine, with favorable neurological recovery. Pediatric NMO is a rare but severe neuroinflammatory disease with variable clinical and immunological profiles. Early recognition through MRI and antibody testing is essential for prompt treatment. High-dose corticosteroids combined with immunotherapy can lead to favorable visual and neurological outcomes.