In patients, CLPP mutations cause complete infertility due to primary ovarian insufficiency and non-obstructive testicular azoospermia, together with early sensorineural progressive deafness, sometimes followed by ataxia and leukodystrophy, inherited in an autosomal dominant manner, a condition known as Perrault syndrome type 3 (PRLTS3) [14,15]. Here, CLPP is linked to Infertility.