In conclusion, this study has identified a novel homozygous missense variant, c.3291 A>C: p.(Asn1097Lys), in CDK13 segregating in a Pakistani family with severe to profound SNHI, bicuspid aortic valve, diabetes mellitus and insipidus, clinodactyly, and GI tract abnormalities which suggests that besides CHDFIDD, CDK13 also likely causes an AR Wolfram-like syndrome which includes cardiac defects, clinodactyly, and GI tract abnormalities. This evidence concerns the gene CDK13 and Wolfram-like syndrome.