The clinical triad of peripheral cutaneous erythematous nodules, oligo or polyarthritis with intraosseous fat necrosis in the setting of pancreatic disease defines a rare entity called pancreatitis-panniculitis-polyarthritis syndrome. The early recognition of this triad is critical due to its high mortality rate and the rapid onset of osseous and articular disabilities. We describe the clinical course of a 54-year-old patient with complaints of weight loss and fever who presented to our hospital with signs of polyarthritis and appendicular erythematous cutaneous nodules. Clinical investigation revealed high inflammatory and pancreatic enzymes levels. Cutaneous biopsy and articular MRI showed evidence of peripheral necrosis. After a thorough investigation, a diagnosis of panniculitis-polyarthritis-pancreatitis syndrome was established. Treatment was initiated, and a slow but steady improvement was observed. Further complications of the disease process were observed. This case highlights the importance of recognising the association between panniculitis and polyarthritis with pancreatic disease in order to improve outcomes.