Titin is the largest known protein with a molecular mass of up to 4,200,000 kDa. Its giant filamentous polypeptide chain forms an intrasarcomeric Z-disc to M-line spanning structure. Within the sarcomere, titin senses strain signals and provides biomechanical feedback to the myocyte. Recent progress indicates that mutations in the titin gene are a frequent cause for hereditary myopathies. In this presentation, recent progress on titin´s medical genetics and potentially emerging treatment strategies will be discussed.