Klinefelter's syndrome and primary central nervous system lymphoma

Klinefelter's syndrome is known to be associated with various malignancies including male breast cancer and germ cell tumour. We report a patient with Klinefelter's syndrome who developed a primary B-cell lymphoma of the central nervous system (CNS). To the best of our knowledge, this is the first case of primary CNS lymphoma in Klinefelter's syndrome.