BACKGROUND : Previous studies strongly suggested the absence of cortical involvement in neuromyelitis optica spectrum disorder. METHODS : We describe two patients with anti-aquaporin-4 antibody and cortical lesions. RESULTS : A 58-year-old and a 61-year-old woman presented with status epilepticus and right leg numbness, respectively. Double inversion recovery (DIR) and phase-sensitive inversion recovery (PSIR) imaging, magnetic resonance imaging sequences that enable the clear delineation of gray matter, revealed intracortical lesions and lesions located across the cortex and subcortex. CONCLUSIONS : Although rare, cortical involvement may exist in aquaporin-4 antibody-positive patients. DIR and PSIR MRI can help to determine the exact location of the lesion.