Primary choriocarcinoma (PCC) of the lung is extremely rare, with about 25 cases previously reported. We describe here a patient with PCC of the lung and review previously described cases to establish the clinical characteristics and most appropriate management of this disease. Survival for patients treated with both surgery and chemotherapy was longer than for patients treated with surgery alone, chemotherapy alone, or optional supportive care. Multivariate analysis of prognostic factors revealed that treatment with both surgery and chemotherapy had independent prognostic significance. Our analysis including previous cases suggests that resection followed by adjuvant chemotherapy is the most effective treatment.