Endomyocardial fibrosis is a rare cardiomyopathy characterized by thickening of the endocardium, which leads to a restrictive phenotype. Differential diagnosis with other forms of cardiomyopathy may not always be straightforward and various imaging modalities are frequently necessary. In the present case, we report a patient with heart failure of uncertain origin, in whom, after an in-depth instrumental evaluation, a rare variant of endomyocardial fibrosis was diagnosed.