Current evidence suggests that almost half of all children with cardiomyopathy and symptomatic heart failure will die or require a cardiac transplant within 5 years of diagnosis. The recognition, diagnostic assessment, and treatment of heart failure in children are therefore challenging undertakings, to say the least. It involves an assessment of cardiac appearance and function, adaptation of the child as a whole, and a diagnostic approach that evaluates many possible root causes. This review is intended to assist the practicing pediatrician and cardiologist by providing a framework for this diagnostic assessment and to give an overview of the treatment options available for children with heart failure. In this first part, we will focus on clinical evaluation, diagnostic testing, and initial medical management. In the second part of this series, the maintenance treatment and treatment options applicable when medical treatment is insufficient will be addressed.