Thymolipoma is an uncommon benign thymus lesion, with a partially deciphered etiopathogeny, being most frequently diagnosed in young patients, regardless of gender. Incidentally diagnosed in asymptomatic patients, larger thymolipomas lead to symptoms related to neighboring mediastinal structures compression, with an intensity which is correlated with the mass size. Our review presents the main epidemiological, pathogenic, clinicopathological and morphological characteristics of this rare pathology. Sometimes, thymolipomas may be associated with paraneoplastic syndromes, which are alleviated by the mass complete surgical resection. Imagistics may orientate the diagnosis, which is certified by the microscopic examination of the resection specimens. Extensive thymectomy remains the current therapeutic option and new tools have been developed to increase the accuracy of the surgical procedure to avoid incidental lesions of the important elements of the anterior mediastinum. Although rare, thymolipomas should be considered in the differential diagnosis of mediastinal masses and of paraneoplastic syndromes.