A previously undescribed case of right ventricular aneurysm (RVA) associated with hypertrophic cardiomyopathy in an advanced stage is reported. The diagnosis was established by noninvasive (cardiac two-dimensional echocardiogram and nuclear MRI) and invasive (cardiac catheterization, angiography, and biventricular endomyocardial biopsy) cardiac examinations, which documented hypertrophied, dilated and hypokinetic biventricular chambers associated with typical histologic findings (histologic hypertrophic cardiomyopathy index of 66%). A prominent narrowing of myocardial arterioles, extended to the right ventricular myocardium, has been identified and has been hypothesized as being responsible for RVA formation.