Primary central nervous system lymphoma (PCNSL) is an uncommon neoplasm, representing 1% of primary brain tumors. PCNSL represents 3% of intracranial neoplasms in patients with AIDS. The aim of this paper will be to review the clinical case reports so far available of pituitary lymphomas in order to depict with the most updated information the clinical syndrome correlated. We report 17 cases of primary pituitary lymphoma (PPL) and 12 cases of secondary localization. The clinical presentation of PPL may be similar to the pituitary invasive macroadenomas. As many as 50% of patients showed evidence of anterior pituitary hipofunction. Of the patients with anterior pituitary failure, 5 had diabetes insipidus; 3 had hyperprolactinemia; 63% had diffuse B cell-large lymphoma. PPL seems to have a better prognosis with respect to all PCNSLs (70% of the examined patients had survived quite long). The data reported suggest that PPLs represent the final presentation of different processes with either hypophyseal or extrahypophysealorigin. Neurosurgical intervention or biopsy are at this point considered crucial for early diagnosis of the tumor
