Pancreatic lipomatosis is the replacement of pancreatic parenchyma by adipose tissue. It is the most common pancreatic lesion encountered, and usually of little clinical significance. Although the exact pathobiology is not known, there are several associated risk factors, such as pancreatitis, obesity, and congenital syndromes. We describe a patient who was previously diagnosed with acute myelogenous leukemia, underwent induction chemotherapy without bone marrow recovery. On presentation to our institution, he was incidentally found to have near complete fatty replacement of the pancreas on computed tomography imaging studies which prompted further evaluation for pancreatic sufficiency. He was subsequently diagnosed with shwachman diamond syndrome. It is necessary for adult physicians to recognize shwachman diamond syndrome as a possible cause of pancreatic lipomatosis, particularly in patients who have cytopenia and hypoplastic bone marrow.