Pulmonary artery dissection is a rare but life-threatening event, predisposing to sudden cardiac death or cardiogenic shock. It is often associated with underlying congenital disorders predisposing to pulmonary hypertension. Rarely, it is diagnosed by echocardiography or other image techniques. We present a case report of a pulmonary artery dissection, diagnosed primarily by echocardiography. The patient died soon after refusing any interventional approach after a short period under medication.