Choriocarcinoma is a malignant tumor that typically appears in gonadal organs and primarily occurs in women of reproductive age. Being a primary extragonadal choriocarcinoma, primary pulmonary choriocarcinoma (PPC) is an extremely rare condition. Due to the rarity of PPC, no standardized treatment has been established so far. However, surgery combined with adjuvant chemotherapy appears to be the most optimal treatment. Here, we report a rare case of a man with PPC that was successfully treated with surgery followed by chemotherapy.