<h4>Objective</h4>To report two cases of aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) with syncope and to review previously documented cases, aiming to enhance recognition of this neurological manifestation and guide timely intervention.<h4>Methods</h4>We reported two NMOSD cases presenting with syncope. Here, AQP4 is linked to neuromyelitis optica.