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Hypertrophic cardiomyopathy (HCM) is the most prevalent (0.2%) heritable, genetic cardiovascular disease. Aortopathies also represent a spectrum of familial inheritance with prevalence of ~4%. Previous research evaluating characteristics of the aorta in HCM patients have demonstrated increased vascular stiffness and differences in aortic elastic properties compared to healthy controls. However, a prevalence of aortopathy in HCM patients has not been previously described and a potential relationship between HCM and aortic dilation has not been established limited by relatively small patient cohorts. We report the case of a 64 years old patient who was admitted to our cardiology department for the treatment of obstructive hypertrophic cardiomyopathy associated with anevrysmal dilation of the ascending aorta.