Abstract Congenital absence of the pericardium is a rare condition. The most common form is complete absence of the left pericardium and asymptomatic patients are discovered during cardiac surgery for an unrelated condition and symptomatic patients may experience chest pain or dyspnea. A 37 year-old female presented with dyspnea and during echocardiography with positional changing maneuver. The patient was found to have complete absence of the left pericardium that confirmed with CT scan. Echocardiography with positional changing maneuver can be diagnostic helpful tool for diagnosis of congenital complete absence of the left pericardium. Keywords: Congenital heart anomaly, Echocardiography, Case report.