Gestational choriocarcinoma is the most common gestational trophoblastic neoplasia. It is prone to hematogenous metastasis, most commonly to the lungs. With the advent and development of chemotherapy, choriocarcinoma has become a curable tumor. However, patients with drug-resistant and recurrent choriocarcinoma are difficult to treat, even with the management of pulmonary metastasis. Resorting to surgery is also a tough decision given the challenges of identifying the appropriate surgical indication and timing. This review discusses the basic principles of management as well as recent advances in the diagnosis and treatment of patients with pulmonary metastasis of gestational choriocarcinoma.