Anaplastic lymphoma kinase (ALK)-rearranged lung adenocarcinoma is generally known to be resistant to erlotinib. We report a case of lung adenocarcinoma with a rearranged ALK and wild-type epidermal growth factor receptor (EGFR) that had good response to the treatment of erlotinib. A 46-year-old man was admitted to our hospital due to severe hypoxemia. Chest CT showed a tumor shadow in the right lung with multiple small nodules. We performed fiberoptic bronchoscopy and diagnosed lung adenocarcinoma (cT4N3M1a, Stage IV). EGFR mutations were analyzed by using polymerase chain reaction (PCR) method, resulted in wild-type EGFR. Another assessment revealed the positive ALK rearrangement, using the methods of immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH). The patient was treated with cisplatin (CDDP) and gemcitabine (GEM); however, the lung tumor was enlarged. After failure of the treatment with docetaxel (DTX), erlotinib was administered as the third-line chemotherapy, leading to the decreased carcinoembryonic antigen (CEA) levels and the drastic reduction of tumor size. Despite the favorable response to this drug, the tumor was again enlarged; thus chemotherapy using bevacizumab was started and continued until tumor regrowth was observed a year later. Erlotinib was then readministered to this patient, resulting in the recovered ability of this drug to reduce the tumor size. The effect has been surprisingly lasting for eight months on the inhibition of tumor growth.