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Fragile X mental retardation protein (FMRP) is a selective RNA-binding protein that is highly expressed in neurons and influences cytoskeletal remodeling, cell-cell signal transduction and interactions. Patients with fragile X syndrome display FMRP deficiency and a lower risk of cancers. However, the specific function of FMRP and the underlying mechanisms are not clear. Evidence indicates the involvements of FMRP in multiple processes of malignant development, including tumor cell migration, invasion, apoptosis, proliferation and metastasis. In addition, FMRP is associated with the occurrence, development and prognosis of tumors. In this paper, we review the progress on the role of FMRP in the pathogenesis of tumors, exploring more potential biomarkers of tumors and new therapeutic targets.