Endomyocardial fibrosis is a disease of unknown cause, characterized by the development of restrictive cardiomyopathy. Although it is endemic in Africa, some cases have been described in Asia, South America and Europe, where a substantial unfamiliarity with this disease still exists. Moreover, differential diagnosis of endomyocardial fibrosis with other cardiomyopathies can be difficult, especially in asymptomatic patients and in initial stage of the disease. After initial echocardiographic analysis, the gold standard imaging technique is the cardiac magnetic resonance. Adjunctive diagnostic tools as endomyocardial biopsy can be considered in ambiguous cases and can help in patient management.