The Kartagener Syndrome is a rare recessive autosomal disease, including the following triad: chronic sinusitis, bronchiectasis, and situs inversus with dextrocardia. The incidence of this genetic disorder is estimated to be about 1/25,000. Our purpose of this case report is to present a 37-year-old woman who was seen by the otorhinolaryngology service of the Hospital das Clinicas, University of São Paulo and who had rhinosinusitis symptoms since childhood, as well as respiratory infections and marital infertility. This report shows the diagnostic methods and their challenges, as well as the recommended treatment, goals, and prognosis of the syndrome. The goal is to begin early treatment of infections of the upper respiratory tract and middle ear and prevent the development of complications such as hearing impairment and bronchiectasis. Prognosis is dependent on early diagnosis and appropriate clinical follow-up.