BACKGROUND Anaplastic sarcomatoid carcinoma of the pancreas (ASCP) is a rare variant of pancreatic malignancies. It is a high-grade epithelial carcinoma predominated with spindle cells. CASE REPORT We report a case of a 65-year-old patient who presented with early stage, ampullary mass indicating malignancy, and who subsequently underwent pancreatoduodenectomy. Histopathology and immunohistochemistry were confirmatory for anaplastic, grade IV sarcomatoid adenocarcinoma arising from the head of the pancreas. CONCLUSIONS To our knowledge, this is a rare presentation with few cases reported in the literature.