The authors present a case of a thirty-nine-year-old white man in good health who developed episodes of ventricular tachycardia as a first manifestation of a right ventricular myxoma, which was diagnosed by two-dimensional echocardiogram and then resected with no complications and total disappearance of the cardiac arrhythmia. After reviewing the literature they consider the present case as a rare manifestation of an infrequent location of an uncommon disease.