Collagen VI-related muscular dystrophies (COL6-RD) are a family of rare diseases associated with collagen VI deficiency, such as the severe Ullrich Congenital Muscular Dystrophy or the intermediate forms of Bethlem Myopathy. The life quality of patients with COL6-RD is highly reduced, due to disabling symptoms, such as respiratory insufficiency, weakness, loss of independent mobility, and joint contracture. Overall, life expectancy is highly decreased, but, to date, there is no effective therapy. Dominant or recessive mutations in one of the three collagen VI genes are considered to be the onset of these diseases. The integrity of the fibrillar network of the collagen VI is lost, altering the incorporation in the extracellular matrix (ECM) and disrupting tissues homeostasis.