Malignancy in patients with sarcoidosis occurs in 3 settings. The first setting relates to patients with hematologic malignancies. It includes the sarcoidosis-lymphoma syndrome, which refers to the development of lymphoma at least 1 to 2 years after the diagnosis of sarcoidosis. It also includes patients with sarcoidosis who develop other hematologic malignancies. In addition, this subset of individuals includes patients with cancer and hematologic malignancies who subsequently develop sarcoidosis. The second setting consists of patients with sarcoidosis who develop solid tumors and oncologic patients in whom sarcoidosis subsequently appears; in addition to melanoma and nonmelanoma skin cancer, the neoplasms most commonly associated involve the cervix, liver, lung, testicles, and uterus. The third setting of malignancy-related sarcoidosis occurs when sarcoidosis presents as a paraneoplastic syndrome for the associated cancer, specifically when the discovery of cancer is concurrent with or within 1 year of the diagnosis of sarcoidosis or vis-a-vis. Antineoplastic treatment of either the hematologic malignancy or the solid tumor has also been observed to either induce the initial onset or flare the activity of sarcoidosis. Malignancy can also be associated with the occurrence of sarcoid reactions that typically are restricted to the regional lymph nodes or the visceral organ of tumor origin; rarely, the sarcoid reaction can also be observed in the skin or is only limited to the skin.