Cholesterol granuloma (CG) is a rare condition histological consisting of a foreign body, giant cell reaction to cholesterol crystals and haemosiderin derived from the ruptured of the erythrocytes. A 25-year-old man came to our Department presenting signs and symptoms of tympano-mastoid cholesterol granuloma. He showed all the specific sign and symptoms of the disease. However, considering the lack of literature regarding TMCG, this study was performed with the aim of presenting the main characteristics of tympano-mastoid CG, describing the case report and reviewing the literature.