Sarcoidosis may present with symptoms related to involvement of joints, skin and lungs, as well as other organs. Corticosteroids are the basis of treatment of symptomatic sarcoidosis. Additional or alternative drugs may be required in chronic cases and when systemic corticosteroids are contraindicated. Cytotoxic agents seem to be of value in selected patients, but no controlled studies are available, and the published literature consists of small case series. Therefore, no international agreement exists regarding when these drugs should be used in patients with sarcoidosis. Methotrexate is generally the preferred agent for treatment of chronic sarcoidosis when corticosteroids have inadequate efficacy and/or severe adverse effects. In this review we discuss the available literature concerning the treatment of sarcoidosis with methotrexate.