<strong>ABSTRACT. </strong><strong><em>Purpose</em></strong>: To carry out systematic review of literature about diagnostics, severity, and dental treatment considerations of children with Hemophilia A. <strong><em>Methods</em></strong>: The search for articles and other document was conducted through the databases available at the National Library System of the National University of Colombia. The English and Spanish terms used in the search were “pediatric dentistry,” “hemophilia A,” dental care,” “coagulation factors,” and/or “coagulation disorders.” <strong><em>Results</em></strong>: The sample consisted of 29 articles that met the inclusion criteria. Hemophilia A is a congenital disorder caused by a deficiency of coagulation factor VIII. Treatment of hemophilia depends on its severity and focuses on avoiding risking life of children due to preventable bleeding by timely applying local therapies such as tranexamic acid or factor VIII, depending on dental needs. Dental treatment of hemophilia A should be interdisciplinary and must emphasize primary health care, health promotion, and prevention in order to avoid invasive dental treatments that could cause bleeding. In addition, treatments seek to maintain oral health and to provide quality of life to children living with hemophilia A.