Isolated tricuspid valve regurgitation can be caused by either primary valvular abnormalities or secondary to annular dilation. Congenital tricuspid valve regurgitation in the adult is rare and encompasses a heterogenous group of anatomical variants that is often associated with or is a sequela of other congenital heart diseases that poses a unique challenge to the surgical management of these patients. We present a case of primary isolated congenital tricuspid valve regurgitation and successful tricuspid valve repair in an adult.