Oncocytic changes may occur very infrequently in neuroendocrine tumours. To estimate the prevalence, pathological features and clinical behaviour of oncocytic endocrine tumours of the pancreas, we reviewed a series of 227 lesions from two institutions. Eleven cases with predominant oncocytic features were selected, representing 4.85% of the whole series. The morphological features and immunophenotype of such tumours did not differ from conventional endocrine pancreatic tumours, except for the presence of abundant eosinophilic and granular cytoplasm. Anti-mitochondrial antigen was positive in all cases tested, and by electron microscopy, numerous mitochondria were observed in the cytoplasm of tumour cells. The majority of cases were nonfunctioning, and in most cases, pathologic signs of malignancy, leading to a diagnosis of endocrine carcinoma, were observed. In addition, the three nonmalignant cases matched the criteria of well-differentiated tumours of uncertain malignant potential. Nearly 50% of the cases were clinically aggressive, and lymph node and liver metastases were present at the time of diagnosis in a minority of cases. Therefore, oncocytic endocrine pancreatic tumours represent a peculiar morphological and clinical variant characterised by frequent hormonal inactivity and malignant behaviour, which should be considered in the differential diagnosis, especially when dealing with a metastatic lesion.