BACKGROUND Primary hepatic carcinoid tumor (PHCT) is a extremely rare. The authors describe a patient with PHCT and review previously published cases of the disease. METHODS A 75-year-old man, presenting with weight loss and pain in the right upper abdomen, had multiple masses in both lobes of the liver. He was diagnosed as PHCT by radiological examination, laboratory findings with high levels of 5-hydroxyindoleacetic acid (5-HIAA) in the serum and urine, and histological findings including positive staining of tumor cells for Grimelius and chromogranin A. The patient received totally transcatheter arterial chemoembolization (TACE) five times over 27 months; this treatment provided excellent palliation and caused a decrease in urinary 5-HIAA levels. Fifty-three cases of PHCT have been reported in the English-language literature. RESULTS Analysis of these published cases revealed that PHCT occurs in the middle age (mean age = 48.2 years) and is more frequent in females (males/females = 20/33 cases). Of the symptomatic patients, the major findings is abdominal pain, fullness, and/or a palpable mass (56% of symptomatic patients). In contrast, only 2 cases out of 53 presented with symptoms of typical carcinoid syndrome. In most cases, PHCT was detected as a hypervascular lesion by radiological examination. By histological analysis, 80% and 84% of the cases were positive for Grimelius silver stain and immunohistochemically positive for chromogranin A, respectively. Surgical resection is the treatment primarily recommended with an 18% of recurrence rate and a 74% of a survival rate after 5 years. For unresectable and recurrent cases, TACE may be recommended.