Congenital absence of the pericardium (CAP) is a rare disorder in which most patients are asymptomatic. The association between this condition and the development of significant tricuspid valvar regurgitation (TR) has been reported, but the causal relationship between them is not clear. Herein is provided a review of all reported cases of CAP associated with TR, and the potential pathogenetic mechanisms behind this association are discussed. Finally, it is suggested that blunt chest trauma might play be a link between these two isolated findings.