We report a case of a young female patient with congenitally unguarded tricuspid orifice and underdeveloped pulmonary valve leaflets, who had developed severe right-sided congestive heart failure and cardiac cirrhosis early in life. She was admitted to our hospital with deterioration of dyspnea and abdominal tenderness. Hepatocellular carcinoma was diagnosed on the basis of computed tomography findings, high plasma alpha-fetoprotein levels and evidence of chronic hepatitis B viral infection. Transthoracic echocardiogram revealed a mass into the right atrium, considered to be an extension of the tumor.