Pulmonary arterial hypertension is a serious condition, with an estimated prevalence of 4 children over a million. Symptoms of this condition are not specific, leading to a potential delay in the diagnosis. Treatment of pediatric pulmonary hypertension is currently based on adult guidelines, using new targeted therapies, separated in 3 main categories: Endothelin receptor antagonists, prostacyclin and analogues, and phosphodiesterase type 5 inhibitors. These therapies improve symptoms and survival, but neither of them can cure the condition. Various new molecules are being currently tested in children or in development, offering hope to further improve the prognosis of pulmonary arterial hypertension.