We present a rare case of newly diagnosed Evans syndrome associated with lung papillary adenocarcinoma in which the patient showed prompt restoration of blood cell count and long-lasting complete remission of Evans syndrome after lung cancer resection. Detailed investigation led to a diagnosis of Evans syndrome. In the first year of the disease, left lower lung papillary adenocarcinoma was diagnosed. Pulmonary lobectomy and three courses of chemotherapy were performed. Six months after the initial visit, the primary lung cancer and the autoimmune diseases appeared to be well controlled. We hypothesized that our patient's initial presentation of hematological manifestation was a paraneoplastic phenomenon associated to her underlying malignancy. This rare case report illustrates the unique relationship between primary lung cancer and the development of paraneoplastic Evans syndrome.