Primary choriocarcinoma of the lung is an extremely rare condition with a fatal outcome in most patients. We report the case of a 31-year-old woman with cough, chest pain, and hemoptysis, whose chest radiograph displayed nodules in the right upper lobe. After study and an unsuccessful attempt at histological characterization of the lesions, exploratory surgery was indicated. The patient underwent lobectomy and lymphadenectomy after diagnosis of a malignant process in the intraoperative biopsy. The definitive diagnosis was choriocarcinoma. Gonadal involvement was ruled out and the patient was referred to the oncology department for adjuvant therapy. She remains free of disease after 42 months. Although choriocarcinoma of the lung is extremely rare and it is hard to establish a standard treatment, in cases of localized disease, surgical resection followed by chemotherapy seems to be useful and can achieve long survivals.