Anti-myelin-associated glycoprotein neuropathy is now recognized as a distinct clinicopathological entity. Since its initial description over 40 years ago, substantial advances have been made in elucidating its pathogenic mechanisms, standardizing diagnostic criteria, and expanding therapeutic options. Recent discoveries-including the identification of M-protein-negative presentations and the efficacy of BTK inhibitors in MYD88-mutant cases-hold considerable promise for enhancing early detection and guiding optimal treatment selection to eventually improve patient outcomes. Nevertheless, future research in this field must prioritize the validation of robust biomarkers, development of more potent disease-modifying agents, and implementation of precision-medicine strategies.