The syndrome is usually caused by mutations in <i>MECP2</i> gene, which is a nuclear protein that selectively binds CpG dinucleotides in the genome.<h4>Materials & methods</h4>To provide further insights into the distribution of mutations in <i>MECP2</i> gene, we investigated 24 females with clinical characters of Rett syndrome referred to Alzahra University Hospital in Isfahan, Iran during 2015-2017. Here, MECP2 is linked to atypical Rett syndrome.