FAM3C is a member of the FAM3 family. Recently, overexpression of FAM3C has been reported in numerous types of cancer, including breast and colon cancer. Increasing evidence suggests that elevated FAM3C and its altered subcellular localization are closely associated with tumor formation, invasion, metastasis and poor survival. Moreover, FAM3C has been found to be the regulator of various proteins that associate with cancer, including Ras, STAT3, TGF-β and LIFR. This review summarizes the current knowledge regarding FAM3C, including its structure, expression patterns, regulation, physiological roles and regulatory functions in various malignancies. These findings highlight the importance of FAM3C in cancer development and provide evidence that FAM3C is a novel biomarker and potential therapeutic target for various cancers.