Carcinoid tumours are known to occur frequently in the gastrointestinal and respiratory tracts. A primary carcinoid tumour of the kidney is an extremely rare entity and only 40 cases have been reported in the literature. As a consequence, very little is known about its real histogenesis, and its prognosis and clinicopathological patterns are not precisely defined. We report a case of primary carcinoid tumour of the kidney found in a middle-aged woman treated by nephrectomy, along with a thorough review of the literature concerning this kind of neoplasm.