Cutaneous metastasis of an occult malignancy to the umbilicus has come to be known as a Sister Mary Joseph's nodule (SMJN). More than 400 cases of this well-described clinical presentation of advanced gastrointestinal and gynecologic cancers have been reported. Pancreatic cancer presenting as a SMJN is a rare phenomenon. In most series, the pancreas is the source of a SMJN in 7% to 9% of cases. We report a case of pancreatic adenocarcinoma in which the initial presenting sign was a SMJN. We also reviewed the published literature from the last 100 years on this phenomenon by conducting a detailed PubMed search. Including this case, we identified 57 cases of SMJN originating from the pancreas. The clinical patient characteristics with regard to age, male-female ratio, race, and prognosis of these cases were similar to that of pancreatic cancer in general. In contrast, 91% of these cases originated in the tail and body of the pancreas rather than the head of the pancreas. This case emphasizes that pancreatic cancer should be considered in the differential diagnosis of umbilical metastasis.