Sarcoidosis involving the pancreas is a rare occurrence. Isolated cases of localized or diffuse involvement of the pancreas have been reported in the literature. The preoperative diagnosis of this entity is a clinical challenge, and surgical intervention is usually needed to make a definitive diagnosis. We report a patient that presents with a preoperative evaluation suggestive of cholangiocarcinoma. Surgical management involved pancreaticoduodenectomy, which revealed pancreatic sarcoidosis with regional lymph node involvement. An extensive literature review of sarcoidosis of the pancreas is provided, which cites all reported cases in which the presentation warranted surgical intervention.