Annotation.Primary ciliary dyskinesia (Kartagener syndrome) refers to inherited rare diseases, characterized by the reverse location of internal organs, the development of secondary chronic diseases of the bronchi, hypoplasia of the sinuses and the development of chronic rhino-sinusitis. In the article the peculiarities of clinic, diagnostics and treatment of children with the syndrome of Kartagener. A clinical caseof diagnosis of this pathology in a child in the first year of life is presented.Early diagnosis and regular adequate conservative therapy contribute to the slow progression of the disease without signs of bronchiectasis.