With morphological features resembling hepatocellular carcinoma, hepatoid adenocarcinoma of the lung (HAL) is a rare and aggressive subtype of lung cancer with a 5-year survival of only 8%. Most HAL patients present increased serum alpha-fetoprotein (AFP) levels that are commonly related to poor prognosis. Most insights into HAL have come from case series or reports, and TP53 is frequently mutated in HAL. The heterogeneity of HAL complicates the diagnosis. With no established standard, the management of HAL is modeled on that of classical lung adenocarcinoma, although with dismal outcomes. Herein, we reported the effective management of a stage IIIb HAL patient with perioperative chemoimmunotherapy and curative-intent surgery. The patient remained disease-free and minimal residual disease (MRD)-negative for more than one year after surgery.