Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a very rare disease. Echocardiographic diagnosis can be very challenging, especially in both asymptomatic children with no history of cardiac disease or critically ill newborns in the setting of intensive care unit. We report a case of ARCAPA in a neonate with congenital pulmonary airway malformation (CPAM), whose echocardiographic diagnosis was particularly challenging due to the critical status at presentation.