Primary clear cell carcinoma of pancreas is extremely rare. We present a case of a 64-year-old male with a mass in the distal body and tail of the pancreas. He underwent a distal pancreatectomy. The histopathology of tumor cells showed features with abundant clear cytoplasm and prominent cell boundaries. Immunohistochemical analysis of neoplastic cells showed reactions to antibodies against cytokeratin-7 and showed no reactions to antibodies against hepatocyte nuclear factor-1β, carbonic anhydrase 9, synaptophysin, and chromogranin A. The patient was subsequently diagnosed with a primary clear cell carcinoma of the pancreas. This is the first time we have encountered it. We report this rare case and update the current literature of this tumor.