The aim of this systematic review is to synthesize existing evidence on the relationship between anti-Müllerian hormone (AMH) levels and ovarian function in Turner syndrome (TS), with three key objectives: (1) to determine the correlation between AMH levels and ovarian reserve, (2) to evaluate AMH’s predictive value for spontaneous puberty onset, and (3) to assess how prognostic factors like age, karyotype, and hormonal status influence AMH’s clinical utility. This evidence concerns the gene AMH and Timothy syndrome.