A couple with normal phenotype has deafness in both families. To reduce the risk of giving birth to deaf baby, the couple has been advised to receive carrier screening before pregnancy. They both carry a heterozygous variant of SLC26A4, c.225C>G (p.Leu75Leu) subsequently confirmed by Sanger sequencing. However, autosomal recessive non-syndromic sensorineural hearing loss typically results from homozygous variants of SLC26A4 gene. It means that this couple have 25% probability of giving birth to a deaf baby. The genetic counselor gave them advice to receive prenatal diagnosis or IVF treatment. After consideration, they gave consent to third-generation IVF treatment. Now they already have a healthy baby.