The clinical course, non-invasive and invasive diagnostic findings from a 50-year-old patient with endomyocardial fibrosis are reported. The patient died in cardiogenic shock. In Europe, endomyocardial fibrosis is a rare event. An exclusively right ventricular involvement is a seldom manifestation. The clinical, echocardiographic and computed tomographic features are diagnostic; therefore a satisfactory differentiation from other cardiac disorders with restrictive hemodynamic signs is possible. An echodense mass as cavity obliteration, with predominantly apical localization, was present at echocardiography; the computed tomography showed a hypodense area instead of the right ventricular cavity.