Background: Marfan syndrome (MFS) is an autosomal dominant disorder of the fibrous connective tissue caused by mutations in the fibrillin-1 (FBN1) gene on chromosome 15q21. Diagnosis of MFS is made according to the Ghent nosology, which is based on data from European and American populations. The aim of this study was to identify clinical characteristics in Korean patients with MFS and to determine the utility of the computed tomography (CT) angiography to diagnose this multisystemic disorder. Methods: A total of 76 patients who fulfilled Ghent nosology from 1995 to 2009 were enrolled in this study. Of 76 patients, 54 patients were probands and 22 patients were family members who were diagnosed in family investigations. Results: Frequencies of pectus carinatum, pectus excavatum requiring surgery, arm span to height ratio >1.05, arachnodactyly, scoliosis, reduced elbow extension, pes planus, and protrusio acetabulae were 28%, 7%, 22%, 71%, 24%, 18%, 57% and 46%, respectively which were lower than that of the western people. The prevalence of ectopia lentis, apical bleb, striae atrophicae, and recurrent hernia were 31%, 39%, 72%, and 11%, respectively which were similar to that of the western people. Dural ectasia which is important clue to diagnose MFS was noticed in 61 patients (80%). CT angiography alone was sufficient for diagnosis in 25 patients (33%). Among the 51 patients who underwent genetic analysis, 44 patients (86%) showed mutations in the FBN1 gene. A total of 50 patients (66%) underwent cardiovascular surgery at the age of 317 on average (Bentall operation in 24 patients, aortic valve sparing operation in 17 patients, descending thoracic or abdominal aorta replacements in 4 patients, AAA repair in 2 patients, valve operation in 6 patients, and CABG in 2 patients). Conclusion: We found a lower frequency of skeletal manifestation in Korean patients than western patients with MFS. CT angiography and genetic analysis were considerably useful in the diagnosis of MFS.