Introduction:Primary pulmonary choriocarcinoma (PPC) is extremely rare, especially in males. It is characterized by a poor response to therapy and shortened survival times. Here, we report a successful diagnosis and modified treatment for PPC in a male and a review of the literature.Case presentation:This case report describes a 67-year-old male who was discovered to have a left pulmonary mass. The patient underwent a pulmonary lobectomy. Pathological examination showed a poorly biphasic differential tumor. Immunostaining displayed that beta-human chorionic gonadotropin (β-HCG), CD10, and GATA3 were positive, and the increase of postoperative serum β-HCG secretion was also confirmed. Systemic and genital screening was performed, but other abnormal findings were not observed. The diagnosis of PPC was confirmed. Then, the patient received 4 cycles of modified chemotherapy according the condition of his body. The patient has been alive for >13 months without recurrence, and the level of serum β-HCG has already decreased to normal. In addition to reporting this case, we have also summarized the similar previously published cases.Conclusions:Currently, there is no standard treatment for PPC. A rapid and correct diagnosis is necessary. Surgery and modified chemotherapy, based on the physical condition of the patient, may currently be the best therapy for PPC.