A 49-year-old man was admitted to our hospital complaining of a large hepatic tumor with edema in the lower extremities. The patient had suffered from hypertension and bronchial asthma in his twenties and from cutaneous flushing in the face in his thirties. Echocardiography revealed tricuspid valve regurgitation and marked dilatation of the right ventricle. In an exploratory laparotomy, a 15 cm-size hepatic tumor was located in the right lobe with multiple satellite lesions in both lobes of the liver. Peritoneal disseminations were present. The tumor was histologically and immunohistochemically diagnosed to be a carcinoid tumor. The levels of serum serotonin and urinary 5-hydroxyindoleacetic acid (5-HIAA) were found to be significantly high. Imaging modalities and intraoperative findings showed no evidence of any tumors elsewhere. These findings led us to the diagnosis of a primary hepatic carcinoid tumor with carcinoid syndrome and carcinoid heart disease. The patient has been treated with hepatic artery embolization, oral chemotherapy and octreotide acetate. After six months of such treatment, the hepatic tumor has decreased slightly in size, and the levels of serum serotonin and urinary 5-HIAA have dropped.