Purpose of Review <p>This paper's objective is to review systemic endemic mycoses, considering the epidemiology, clinical manifestations, diagnostic methods, and treatment in the pediatric population.</p> Recent Findings <p>The increase in awareness, description, and diagnosis of these diseases in the pediatric population allows us to make a more precise characterization of the clinical manifestations, as well as the description of risk factors.</p> Summary <p>The clinical presentations of these mycosis are varied; high clinical suspicion and geographical background are required for diagnosis. Immunocompromised patients usually present with the most severe manifestations but may present in immunocompetent patients.</p>