The revised Ghent nosology for Marfan syndrome (MFS) aims to improve the accuracy and practicality of diagnosing MFS by emphasizing cardiovascular manifestations, particularly aortic root aneurysm and ectopia lentis. The new criteria are designed to address concerns with the current nosology, such as the lack of validation of some diagnostic criteria and the need for expensive specialized investigations. The revised criteria provide clear guidelines for diagnosing MFS in both sporadic and familial cases, with a focus on distinguishing MFS from related conditions like Sphrintzen–Goldberg syndrome (SGS), Loeys–Dietz syndrome (LDS), and vascular Ehlers–Danlos syndrome (vEDS). The criteria also introduce a new scoring system for systemic features and emphasize the importance of molecular genetic testing, particularly for *FBN1* mutations. The revised nosology aims to reduce the risk of premature or misdiagnosis while facilitating worldwide discussion and standardized risk and follow-up/management guidelines.The revised Ghent nosology for Marfan syndrome (MFS) aims to improve the accuracy and practicality of diagnosing MFS by emphasizing cardiovascular manifestations, particularly aortic root aneurysm and ectopia lentis. The new criteria are designed to address concerns with the current nosology, such as the lack of validation of some diagnostic criteria and the need for expensive specialized investigations. The revised criteria provide clear guidelines for diagnosing MFS in both sporadic and familial cases, with a focus on distinguishing MFS from related conditions like Sphrintzen–Goldberg syndrome (SGS), Loeys–Dietz syndrome (LDS), and vascular Ehlers–Danlos syndrome (vEDS). The criteria also introduce a new scoring system for systemic features and emphasize the importance of molecular genetic testing, particularly for *FBN1* mutations. The revised nosology aims to reduce the risk of premature or misdiagnosis while facilitating worldwide discussion and standardized risk and follow-up/management guidelines.