We report a 51-year-old patient, diagnosed with single left ventricle and anterior rudimentary chamber which was corrected by a Fontan procedure in 1997. The echocardiogram, Holter and pulmonary perfusion scintigraphy, isotopic ventriculography, magnetic resonance and blood tests performed during follow-up showed an uncomplicated postoperative course. The patient remains asymptomatic. We believe that this is the oldest case suffering from this anomaly who underwent surgical repair.