Right ventricular endomyocardial biopsy is difficult to perform in patients who underwent heterotopic heart transplantation because of the complex vascular anatomy. The procedure is usually performed under fluoroscopic control. We present a case of a 59-year-old woman that after heterotopic heart transplantation underwent echo-guided endomyocardial biopsy. We report the technique discussing data obtained in other 11 patients heart transplanted in the heterotopic way. In conclusion we believe that echocardiographic guidance during endomyocardial biopsy allows a better choice of bite sites, reduces the risk of free wall perforation. Moreover is diminished the risk of X-ray exposure to both patient and operator.