Carbamyl phosphate synthase 1 （CPS1） is one of the rate-limiting enzymes in the urea cycle, which is mainly expressed in liver cells and intestinal epithelial cells. Under normal physiological conditions,CPS1 plays an important role in the removal of ammonia, a metabolic waste in the body. Studies have shown that CPS1 gene is intimately related to liver cancer, lung cancer, colorectal cancer and other malignant tumors, and can affect cell proliferation, apoptosis, migration and other cell biological behaviors through a variety of signal transduction pathways or affecting pyrimidine synthesis, thus affecting the occurrence and development of tumors or clinical prognosis of patients. Therefore, analyzing the relationship between CPS1 gene and malignant tumor is of great significance for early diagnosis, treatment and prognosis evaluation of tumor. This article reviews the relationship between CPS1 gene and tumor.