Liver resection for primary hepatic neuroendocrine carcinoma (phNEC) has only scarcely been reported in the literature. We herein report on a 19-year-old female with a solitary 27 × 13-cm-big phNEC, which was initially considered as hemangioma. An extended right hepatectomy (segments V–VIII, partially IVa) was performed. Resection margins were free of tumor (R0 resection). Ki67 expression was 35 %. Postoperative course was uneventful, and the patient was discharged on the seventh postoperative day. Two years after surgery, the patient remains disease-free and in good general condition. Large series and longer follow-up studies are required for the better understanding on this rare tumor entity.
