Carcinoid heart disease is characterized by heart valve dysfunction as well as carcinoid symptomatology. We report a case of carcinoid heart disease associated with a primary ovarian tumor. A 60-year-old woman presented for dyspnea evaluation with a history of facial flushing, telangiectatic skin changes, and pitting edema of both lower extremities. Chest radiography showed cardiomegaly, and echocardiography revealed an isolated, severe tricuspid regurgitation without left-sided valvular dysfunction. The tricuspid leaflets were severely retracted and shortened, resulting in poor coaptation. Furthermore, mild pulmonary valve stenosis and moderate regurgitation were found along with this deformation. The 24-hour urine analysis revealed an increased level of 5-hydroxyindoleacetic acid, and an ovarian tumor was apparent on computed tomography images. The mass was surgically removed, and the patient was diagnosed as having a primary ovarian carcinoid tumor. She was treated with chemotherapy and regularly followed-up with supportive treatments, deferring surgical correction.