Non compaction cardiomyopathy is a rare entity which usually involves the left ventricle causing heart failure, ventricular arrhythmias, peripheral embolism and sudden death. So far, very few cases of right ventricular non compaction have been described, for which no established diagnostic criteria exist and the clinical course as well as the response to treatment of these patients remain unknown. We describe the case of a patient with marked right ventricular hypertrabeculation in whom a structural origin of it was discarded, whereby the diagnosis of right ventricular non-compactation was made.