Isolated ventricular non-compaction (IVNC) is a genetically determined cardiac disease mainly involving the left ventricle. Since the first description and recognition of IVNC as a distinct cardiomyopathy, many cases have been described. However, the associated right-sided cardiac anomalies are unusual. Two cases of IVNC showing associated right-sided pathology on transthoracic echocardiography are presented.