We present a patient with the symptomatic pancreatic disease. Extensive preoperative workup could not reveal the diagnosis beyond doubt, so Whipple’s resection was performed, in order to exclude pancreatic adenocarcinoma. Since the difference diagnosis between autoimmune pancreatitis and pancreatic cancer cannot be made based only on clinical findings, serological and imaging examinations and biopsy is not always feasible, many patients have to undergo surgery. Autoimmune pancreatitis is the most common manifestation of a newly recognized entity, IgG4-related systemic disease, affecting many organs and sites. The second most common manifestation, which patient, also presented with salivary gland enlargement, also known as Kuttner’s tumour, and has histopathologic findings common with autoimmune pancreatitis. The IgG4-related systemic disease can be effectively treated with steroids, so accurate diagnosis, even if is sometimes difficult, can help prevent unnecessary surgery. On the other hand, despite the fact that this entity is not primarily managed by surgery: surgery is often employed for a diagnostic and management purposes.