Tendons and ligaments (T/L) are very similar fibrous tissues that respectively connect muscle to bone and bone to bone. They are comprised of fibroblasts that produce large amounts of extra-cellular matrix, resulting in a dense and hypocellular structure. The complex molecular organization of T/L, together with high water content, are responsible for their viscoelastic properties, hence insuring their mechanical function. We will first review recent work on tendon embryology and discuss ligament formation, which has been less documented. We will next summarize our current knowledge of T/L molecular architecture, alterations of which are a major cause for disease. We will finally focus on T/L repair after injury and on genetic diseases responsible for T/L defects. Birth Defects Research (Part C) 75:226–236, 2005. © 2005 Wiley-Liss, Inc.