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Since 1986, the simultaneous presence of an hematologic malignancy and sarcoidosis, defined as sarcoidosis-lymphoma syndrome (SLS), has been described in 79 patients. The majority of these patients were affected by sarcoidosis and developed, after one or two years, a non-Hodgkin Lymphoma or an acute leukemia, while in only less than twenty cases the malignancy came first. In this report we describe the case of a 83 year old man with a clinical history of gastric mucosa-associated lymphoma (MALT), which was Helicobacter pylori-positive. He developed sarcoidosis ten years after the first diagnosis. The late onset of sarcoidosis in our patient made the diagnostic work-up and differential diagnosis between a lymphoma relapse and a de novo sarcoidosis extremely challenging.