Right ventricular non-compaction cardiomyopathy (RVNC) is a genetic heterogeneous cardiomyopathy. Despite the increasing number of RVNC cases, the classification and natural history of this disorder are not completely clear. Also, because the pathogenic non-compaction cannot be easily differentiated from normal trabeculations, it is usually hard to accurately measure the prevalence of RV involvement. In this review, we searched main databases including Medline, Web of Science, EMBASE, and Scopus. In addition, the Google Scholar was searched until December 2019. Owing to the disagreements over RVNC classification and lack of diagnostic tests, there is no reliable and accurate statistical data about the prevalence of RVNC in newborn and children populations. Hypothetically, it is propounded that the disorder is being over-diagnosed as the number of RVNC cases reported during recent years has significantly increased.