This article reports the diagnosis and treatment process of a patient with retroperitoneal enterogenous cyst with glandular epithelial carcinogenesis. Contrast-enhanced computed tomography performed at initial diagnosis showed right retroperitoneal cystic space-occupying lesions which were likely to be malignant. The patient underwent laparoscopic resection of right adrenal adenoma+nephron-sparing right renal cyst resection+right retroperitoneal cyst resection，as well as four cycles of XELOX chemotherapy after surgery. Reexamination performed half a year later showed recurrence and metastasis of cancerous enterogenous cyst.
