Primary neuroendocrine carcinoma of the liver is a rare tumour, probably arising from scattered neuroendocrine cells of the bile duct. We present the case of a 72-year-old male who experienced gradual weight loss and diarrhoea. Given the fact that he had stayed in the Dominican Republic, a parasitic disease was initially suspected. However, this was not confirmed. Further examination showed tumour infiltration of the liver. Fine needle aspiration cytology of the tumour site was performed. The diagnostic procedure revealed neuroendocrine carcinoma. The tumour cells expressed the following neuroendocrine markers (chromogranin, synaptophysin, CD56 and NSE) as well as the epithelial marker AE1-AE3. The tumour was considered metastasis of the primary tumour located in the gastrointestinal tract. A thorough clinical examination was performed including gastroscopy, colonoscopy, In-111 Octreoscan scintigraphy, computed tomography and magnetic resonance imaging. These methods revealed metastases in the vertebrae, pelvis, long bones and skull. No other tumour sites were found in the lungs, gastrointestinal tract or pancreas. The patient became increasingly cachexic and later died. An autopsy showed massive multicentric tumour infiltration of the liver. Histological examination revealed well differentiated neuroendocrine carcinoma which transformed into intermediate and small cells. The autopsy found no tumour sites in the gastrointestinal tract, lungs or pancreas. The results were suggestive of primary neuroendocrine carcinoma of the liver. BACKGROUND neuroendocrine carcinoma - liver - primary tumour.