A case of Werner's Syndrome in a 47-year-old man, with typical features of progeria associated with intracranial meningioma is described. A revision of the literature showed that meningioma is the most frequent benign neoplasm in Werner's Syndrome. Meningioma is a peculiar model of neoplasm, because of the frequency of cytogenetical aberrations concerning chromosome n. 22. Either chromosome n. 22 and other chromosomal alterations could be detected in peripheral blood lymphocytes of our patient. These findings suggest a correlation between chromosomal instability and the onset of neoplasms in Werner's Syndrome. Furthermore, the possibility of detecting chromosome n. 22 aberrations in peripheral blood lymphocytes of Werner's Syndrome patients could provide a clue to the presence of a meningioma at a preclinical stage.