Transcription factor families are a small number of upstream master genes in "higher hierarchy" that control the expression of a large number of downstream genes. These transcription factors have been found to integrate the signaling pathways underlying the pathogenesis of cardiovascular diseases with or without autoimmune inflammatory mechanisms. In this chapter, we organize our analysis of recent progress in characterization of forkhead (Fox) transcription factor family members in vascular pathology and immune regulation into the following sections: (1) Introduction of the FOX transcription factor superfamily; (2) FOX transcription factors and endotheial cell pathology; (3) FOX transcription factors and vascular smooth muscle cells; and (4) FOX transcription factors, inflammation and immune system. Advances in these areas suggest that the FOX transcription factor family is important in regulating vascular development and the pathogenesis of autoimmune inflammatory cardiovascular diseases.