Lobular panniculitis, together with a polyarthritis may complicate pancreatic disease. Abdominal symptoms are frequently absent and mis-diagnosis of the joint-skin complex can lead to inappropriate treatment in a condition with an already high mortality. We report a case of the pancreatitis panniculitis polyarthritis (PPP) syndrome, complicated by metastatic fat necrosis, with bone marrow involvement and characteristic magnetic resonance imaging (MRI) and describe the clinical characteristics, therapy and outcome of patients affected by the syndrome.