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 PPC case which was complicated by choriocarcinoma syndrome (CS) and a review of the literature.Case presentation: A 59-year-old man with 30-pack-year smoking history was referred to our hospital because of hemoptysis and chest pain. Positron emission tomography-computed tomography (PET-CT) showed multiple bilateral pulmonary nodules and multiple metastases throughout the body. The patient underwent single-port thoracoscopic wedge resection of the right lung upper lobe. Histological examinations verified the diagnosis of choriocarcinoma. Three days after the operation, the patient developed massive hemoptysis. A bronchoscopic examination showed bleeding from the bilateral main bronchus. Despite management in the intensive care unit (ICU), the patient died two weeks after surgery. Conclusions: A rapid and correct diagnosis of PPC is necessary. When the patient has massive hemoptysis, we should be alert to the occurrence of CS. Surgery and modified chemotherapy, based on the physical condition of the patient, may currently be the best therapy for PPC.