Hepatoid adenocarcinoma of the lung (HAL) is an extremely rare cancer without clear treatment guidance and with a poor prognosis. This report discusses a 64-year-old man who presented with complaints of hemoptysis and was found to have a right upper lobe (RUL) lung mass on chest CT with presence of a right hilar mass and retrocaval lymphadenopathy, and metastasis to the vertebral spine and rib. The patient was diagnosed with T2N2M1 (stage IV) unresectable disease. A biopsy of the RUL mass revealed hepatoid variant adenocarcinoma. Immunohistochemical stains showed tumor cells positive for CK7, AFP, Hep Par 1, napsin A, and cytoplasmic TTF-1 staining. In contrast, CK5, CK6, and CK20 were negative, and EGFR was wild-type. Serum alpha fetoprotein (AFP) level was elevated at 181 ng/mL. The patient was treated with platinum-based doublet chemotherapy and sorafenib, and his AFP level decreased to 25 ng/mL. This case report presents the novel use of sorafenib in combination with platinum-based doublet chemotherapy in EGFR wild-type HAL, which led to a partial response. Single-agent sorafenib led to stable disease overall, achieving a survival among the longest reported for unresectable stage IV, all while maintaining an ECOG performance status of 0 to 1.