I have reported the case of a 62-year-old man with chronic alcoholic pancreatitis and a rare pseudocyst-portal vein fistula. Even though he experienced no abdominal symptoms, he had severe metastatic fat necrosis manifested as subcutaneous fat necrosis, polyarthritis, medullary bone necrosis, and mental status changes. Remote tissue destruction continued until relief was gained by pancreaticoduodenectomy and repair of the necrotic portal vein. Disseminated fat necrosis is a rare syndrome that can be the only presenting feature in patients with pancreatitis and pancreatic cancer. Early recognition and treatment of the underlying pancreatic disease may decrease the high morality rate associated with this syndrome.