Duplication of an atrioventricular valve is a rare congenital anomaly that usually involves the mitral rather than the tricuspid valve (TV). Isolated appearances of a double-orifice TV (DOTV) are extremely rare and in most cases are associated with other congenital anomalies. We report a rare case of an HIV-seropositive male with a DOTV, divided right atrium and Wolff-Parkinson-White syndrome admitted to our hospital with palpitations and dyspnea. After medical therapy, the patient's symptoms were alleviated. We summarized the characteristics of the DOTV and reviewed the literature.