AIM: The aim of this study is to demonstrate how surgery is fundamental in case of Kuttner Tumour (KT). In literature, there are few reported cases of KT and for this reason, diagnostic errors could occur with subsequent underestimation of the disease. MATERIALS OF THE STUDY: We review cases of KT published from 1976 to today in order not to run into diagnostic errors. It was carried out a systematic review of the literature on chronic sclerosing sialadenitis, also known as KT. RESULTS: KT is an immune-mediated localized fibro inflammatory condition that often mimics other pathological processes, such as neoplasms. DISCUSSION: The variables analysed in each article included in this review were the age and gender of the patients, the location of the disease, the type of study; clinical presentation, instrumental tests performed, presence of IgG4, surgery performed and the evolution of patients after treatment were also assessed. Diagnosis should be based on clinical, serological and pathological findings, but in a small percentage of cases (just as in the case presented) the cytological data provided by FNAB and serum IgG4 levels do not allow a diagnosis. CONCLUSIONS: Our experience shows that only surgery with subsequent histological examination makes it possible to correctly diagnose the disease.