Tumors of the pancreatic head commonly consist of carcinomas whereas other entities are rare exceptions. Extrapulmonary sarcoidosis is well-known but is extremely rare when detected as a mass in the pancreatic head. In general the diagnosis of sarcoidosis requires histologic examination with verification of non-caseous, epithelioid cell-like granulomas. Systemic therapy consists of steroids when the patient exhibits symptoms or in the case of progression of the disease. However, in some cases extended abdominal resections are also required to confirm the diagnosis and/or to treat symptoms.