The small GTPases of the Rho subfamily (RhoA, Rac1 and Cdc42) are signaling molecules involved in cytoskeleton remodeling and gene transcription. Their activities are important for many cellular processes, including myogenesis. Classical cadherin adhesion molecules are key determinants of cell recognition and tissus morphogenesis and act as adhesion-activated signaling receptors. Rho GTPases have emerged as key mediators of their activity. Not only signal transduction pathways link cadherins to Rho GTPases but also Rho GTPases to cadherins. We focus in this review on the role of cadherins and Rho GTPases in normal myogenesis as well as in pathological development of rhabdomyosarcoma.