Retinoic acid (RA), a lipid soluble signaling molecule derived from vitamin A (retinol), regulates diverse biological processes, including cellular proliferation, differentiation, and apoptosis, throughout embryonic development. RA controls the expression of genes involved in patterning and morphogenesis during organogenesis. Disruptions in the regulation of RA signaling results in several developmental disorders, including limb and skeletal defects, abnormal patterning of the central nervous system, ocular and craniofacial defects, cardiac malformation, foregut endoderm defects, and renal agenesis. Identification of pleiotropic functions for RA during organogenesis has provided a better understanding of cellular and molecular events controlling embryonic development. Loss-of-function studies using mutants of RA-synthesizing enzymes and RA receptors (RARs) have been particularly helpful in unraveling the mechanism of RA signaling. Further studies using genetic models are required to fully understand the molecular logic of RA signaling from embryogenesis to adulthood.