The combination of pancreatitis, panniculitis, and polyarthritis (PPP) is a rare systemic syndrome that occurs in patients with acute or chronic pancreatitis or pancreatic malignancies. A 50-year-old male patient presented with polyarthritis increasing for 1 week and consequent inability to walk unaided. In addition, the patient had several isolated nodules on the lower extremities without any tenderness to pressure. Laboratory tests showed elevated pancreatic enzymes indicative of pancreatitis, which was thereafter confirmed by abdominal CT scan, with signs of chronic pancreatitis and concrements in the pancreatic duct. The diagnosis of panniculitis was established by dermatological consultation. Considering all these clinical features, PPP syndrome was diagnosed. In accordance with the pre-existing literature, oral glucocorticoid therapy and nonsteroidal anti-inflammatory drugs (NSAIDs) were given but failed to improve pain and polyarthritis. In the further clinical course, due to the persistent increase in lipase and amylase, endoscopic retrograde cholangiopancreatography (ERCP) was performed, showing small concrements in the pancreatic duct. During the intervention, the pancreatic duct was widened, the small concrements were removed, and a pancreatic duct stent was then implanted. Following ERCP, an instant decrease in pancreatic enzymes was observed, accompanied by a clear and sustained improvement of joint swellings and pain. With the typical triad of clinical findings in mind, one should consider PPP syndrome in the context of acute or chronic pancreatitis as well as in pancreatic malignancies. All involved disciplines (gastroenterology, dermatology, and rheumatology) should be familiar with this rare but severe condition. The prognosis depends on the extent of the functional deficit of the extremities and the progression of the underlying disease.