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Childhood idiopathic pulmonary arterial hypertension (CIPAH) represents a rare disorder of childhood characterized by dyspnea, exercise limitation, and syncope. Common organ-specific pathology includes right ventricular myocardial hypertrophy. The case presented represents a classic case of CIPAH, where death was partially attributable to an unrecognized esophageal intubation, which was placed during emergency transport to an emergency department. The features of CIPAH are presented, along with a discussion related to esophageal intubation and death.