The Dementia with Lewy Bodies (DLB) Consortium has updated its consensus criteria for the diagnosis and management of DLB, distinguishing between clinical features and diagnostic biomarkers. The revised criteria emphasize the importance of REM sleep behavior disorder (RBD) and 123I-iodine-metaiodobenzylguanidine (MIBG) myocardial scintigraphy. The criteria also incorporate new information on pathologic heterogeneity and provide guidance on optimal methods for establishing and interpreting these markers. The clinical management of DLB is complex, requiring a multidisciplinary approach, and the recommendations are based on expert opinion due to limited randomized controlled trials. Key areas of future research include developing guidelines and outcome measures for clinical trials, characterizing biological mechanisms, and advancing research through global harmonization efforts.The Dementia with Lewy Bodies (DLB) Consortium has updated its consensus criteria for the diagnosis and management of DLB, distinguishing between clinical features and diagnostic biomarkers. The revised criteria emphasize the importance of REM sleep behavior disorder (RBD) and 123I-iodine-metaiodobenzylguanidine (MIBG) myocardial scintigraphy. The criteria also incorporate new information on pathologic heterogeneity and provide guidance on optimal methods for establishing and interpreting these markers. The clinical management of DLB is complex, requiring a multidisciplinary approach, and the recommendations are based on expert opinion due to limited randomized controlled trials. Key areas of future research include developing guidelines and outcome measures for clinical trials, characterizing biological mechanisms, and advancing research through global harmonization efforts.