Pathogenic variants in genes that encode the skeletal sarcomeric proteins tropomyosin (TPM2; MIM: 190990);4 troponin I2, fast skeletal type (TNNI2; MIM: 191043);4 troponin T3, fast skeletal type (TNNT3; MIM: 600692);5 myosin heavy chain 3 (MYH3; MIM:160720);6 and myosin heavy chain 8 (MYH8; MIM: 160741)7 account for most cases of distal arthrogryposis (DA), a group of Mendelian conditions characterized by non-progressive congenital contractures of the limbs and, less frequently, contractures of the face, strabismus, neck webbing, pterygia, short stature, and scoliosis. This evidence concerns the gene TNNI2 and distal arthrogryposis.