The congenital atriquia with papular lesions is a rare genetic disorder of alopecia with a surprisingly low frequency. It is caused by mutation in the hairless gene of chromosome 8p12 with an autosomal recessive pattern. Reported a case of female patient, a 36-years-oldwoman was referred to the of ce of Medical Genetics in the Faculty of Health Sciences at the University Centrocciodental “Lisandro Alvarado”. The woman presented alopecia and papular lesions. The ndings are discussed, the literature is reviewed, and patient’scharacteristics are compared with those found in other cases. The diagnosis is congenital atriquia with papular lesions.