The incidence of combined hepatocellular carcinoma-cholangiocarcinoma (cHCC-CC) in a single patient accounts for only 0.4 to 14% of all primary liver cancer. However, the prognosis of its intrahepatic cholangiocarcinoma (ICC) component is poor. We experienced a unique case of a sequentially developed cHCC-CC with adrenal metastasis as the primary presentation and a hidden primary hepatocellular carcinoma. A 65-year-old female with a history of jaundice and abdominal discomfort was diagnosed with S4 ICC measuring 5 cm in diameter, and characterized histologically as papillary adenocarcinoma with intraductal growth, but without any evidence of malignant hepatocyte. S4 segmentectomy with hepaticojejunostomy revealed no additional masses. A follow-up CT scan 3 months after surgery showed a right adrenal mass with markedly increased serum AFP (4950 ng/mL), which was treated with right adrenalectomy. Histopathology revealed a metastatic hepatocellular carcinoma testing positive for AFP, glypican-3, and hepatocytes, but negative for CD-10, inhibin-α, EMA, S-100, and cytokeratin-7. Serum AFP level immediately plummeted to 4.1 ng/mL upon adrenal mass removal. A recurrent S7 liver mass was suspected 1 year later with serum AFP value of 7.6 ng/mL, and characteristic CT imaging of HCC. TACE was performed with good response. Adrenal metastasis may manifest as the primary focus of hepatocellular carcinoma in sequentially developed cHCC-CC patients with hidden primary HCC. cHCC-CC should be considered in the differential diagnosis of cholangiocarcinoma with elevated AFP.