KIAA1199, also known as CEMIP or HYBID, is an important member of the Human Unidentified Gene-Encoded (HUGE) database. Accumulated evidence has revealed that KIAA1199 is associated with tumor progression and metastasis in numerous malignancies, including colorectal, liver, gastric, pancreatic, breast, lung, prostate, ovarian and papillary thyroid cancers. As an oncogene, it plays crucial role in the proliferation, apoptosis, invasion and migration of various tumor cells. In addition, KIAA1199 is also involved in the regulation of multiple signal pathways such as epithelial-mesenchymal transition (EMT), Wnt/ β-catenin, MEK/ERK and PI3K/Akt. In this review, we summarized up to date advancement on the role of KIAA1199 in human cancer development, progression, and metastasis. We also addressed KIAA1199 as a potential therapeutic target for cancer therapy.