Ninety percent of all carcinoid tumors develop in the gastrointestinal tract. Although the liver is a usual site for metastases, primary hepatic carcinoid tumors (PHCTs) are extremely rare. The diagnosis is based on histopathologic characteristics and on exclusion of a nonhepatic primary tumor. While liver transplantation (OLT) is a well-established surgical treatment in selected cases of unresectable metastatic carcinoid tumor, its use in PHCT has not been widely described. We report the case of a 50-year-old woman with unresectable PHCT treated with OLT. After 64 months, disease recurred in the liver and mesentery. Laparotomy with multiple radiofrequency ablations of liver lesions and resection of peritoneal deposits was performed; however, in the postoperative period, a fatal myocardial infarct occurred. Our case is the fourth one reported in literature. It confirms long-term survival after OLT in patients with unresectable PHTCs.