A single ventricle is a congenital malformation including heart defects that results in having one functional ventricle and another small or hypoplastic one. A new concept of this congenital anomaly defines it as two ventricles unable to sustain separately systemic and pulmonary circulation.

We report a rare case of an 18-years-old female surviving a single ventricle malformation associated with a single atrium and a situs inversus viscerum. She was misdiagnosed in utero and at birth and lost to follow up since her early age when diagnosed at 4-years-old and didn't undergo any palliative surgeries after then due to financial issues. Patient was admitted for heart failure decompensation with hepatic congestion, supraventricular arrhythmias and bilateral pleural effusion and was treated symptomatically. This described patient is a very rare congenital association with a bad prognosis and low survival rate.

Early diagnosis and follow up in these congenital cases are important to improve quality of life and life expectancy.