Forkhead box protein C2 (FOXC2), a transcription factor of the forkhead/winged-helix family, is required for embryonic and prenatal development. FOXC2 acts as a crucial modulator during both angiogenesis and lymphangiogenesis via multiple angiogenic and lymphangiogenic pathways, respectively. Although recent studies have shed light on the emerging role of FOXC2 in cancer, very little is known about the precise underlying mechanisms. The purpose of this review is to summarize the current understanding of FOXC2 and provide potential mechanistic explanations of the relationship between FOXC2 and cancer, as well as discuss the prospect for future research in the promising prognostic value of FOXC2 in cancer.