Single ventricle is a rare congenital anomaly that accounts for about 1.5% of all congenital heart defects at birth. It is believed to carry a poor prognosis when untreated, which has led to a more aggressive surgical approach in childhood. The surgical approach is an initial palliative operation in the form of a shunt, followed by a Fontan operation or one of its modifications. However, a few case reports have noted the survival of patients to the fifth or sixth decade without surgical repair. We experienced an anesthesia of a 20-year-old female patient for excision of the pheochromocytoma who had chronic hypoxia due to partially corrected single ventricle anomaly. We report this experience with brief review of literature.