Marfan's syndrome is an autosomal dominant disorder of connective tissue characterized by a large number of possible mutations and by heterogeneity of clinical presentation primarily in skeletal, ocular and cardiovascular organ systems. Cardiovascular complications of the disease are responsible for high mortality. The case of a 42-year-old patient with a progressive advanced abdominal aorta dilatation visualized on computed tomography images is presented. Pathogenesis, diagnosis and management of patients with Marfan's syndrome are also discussed.