A recurrent pathogenic variant, <i>MLH1</i> c.306G>T; p.(Glu102Asp), accounted for nearly one-quarter of Lynch syndrome cases and was observed exclusively in individuals of Punjabi ancestry, suggesting a strong founder effect.<h4>Conclusions</h4>Lynch syndrome contributes substantially to colorectal and endometrial cancers in the North Indian population. This evidence concerns the gene MLH1 and endometrial cancer.