Choriocarcinoma is a malignant tumor associated with early vascular invasion and a high mortality. Ultra-high-risk choriocarcinoma, which was proposed in the International Federation of Gynecology and Obstetrics cancer report of 2018, has a higher risk of treatment failure and a worse prognosis than choriocarcinoma. We report a rare case of a 39-year-old female patient with ultra-high-risk choriocarcinoma (stage IV:20) with hemorrhage secondary to atraumatic splenic rupture as the initial sign. A satisfactory outcome was achieved through comprehensive treatment with surgery, chemotherapy, immunotherapy, and targeted therapy.