At present, more than 150 ectodermal dysplasia have been described, many of which may have similar clinical manifestations. Differential diagnosis of the clinical picture of ectodermal dysplasia is an important aspect in the examination of the patient and is carried out to clarify the diagnosis, to select in the future the optimal method of molecular genetic research and medical and genetic counseling of family members. We present a clinical observation of a rare form of ectodermal dysplasia in a child 1 year old with incomplete Hays-Wells syndrome due to a mutation in the TP63 gene. This case is the first observation of Hays-Wells syndrome in a child described in Russian practice. Clinical symptoms typical for this syndrome are described: hypotrichosis with poorly healing scalp erosions, as well as other skin and extra skin manifestations. In a microscopic examination of a child’s hair, anomalies of the hair shaft in the form of twisted hair and so-called pili canaliculi were revealed. These anomalies belong to the pathology accompanied by increased hair brittleness, which leads to the development of hypotrichosis. The diagnosis is confirmed by molecular genetic studies. As a result of exzom sequencing in the TP63 gene in heterozygous state, the replacement of rs121908843 NM_003722.4: c.1681T>G was found, resulting in the replacement of amino acid p.Cys 561Gly.