Hepatoid carcinoma (HC) is an extremely rare form of neoplasm. Its cellular structure resembles that of a hepatocellular carcinoma (HCC). To date, only 26 cases of hepatoid carcinoma of the pancreas have been reported in the literature. We report the diagnosis of a hepatoid carcinoma of the pancreatic head in a 78-year-old male patient. The tumor was detected incidentally during routine abdominal ultrasound scanning. Laboratory tests did not show any abnormalities except for a monoclonal gammopathy of undetermined significance. After CT, MRI, and laparoscopic biopsy that failed to obtain the diagnosis, the patient underwent a Whipple procedure. The final pathology report described a hepatoid carcinoma of the pancreatic head (pathological T3, N0 (0/10), L0, V0, R0, M0). After the patient recovered, no further therapy was recommended by the tumor board and he was discharged. Regular follow-up was suggested; however, the patient suddenly died of acute coronary artery disease 2 months after surgery.