Abstract: Objective We report a rare case of a multiple sarcomatoid carcinoma of the jejunum with postoperative lung and brain metastases. The patient underwent jejunum segmental resection for intussusception and gastrointestinal bleeding. Multiple metastasis ofbrain and lung occurred 4 months after the operation, and the patient died for multiple organ failure 8 months after the surgery. Primary sarcomatoid carcinoma was difficult to diagnose at an early stage, and the diagnosis relies on optical microscopic and immunohistochemical observations. Currently no guidelinesare established for treatment for sarcomatoid carcinoma of the jejunum, and surgical resection remains the optimal therapeutic approach. Previous reports documented a poor prognosis of the patients with a median survival time of 5.5 months (0.36- 36months), and the primary causes of death were tumor recurrence and metastasis. Ki-67 as a potential prognostic marker and the value of PD-L1-targeted immunotherapy for the treatment await further investigation.