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Persistent left superior vena cava (PLSV) is an abnormality of drainage of the embryological left cardinal vein, persisting as left superior vena cava and commonly emptying into the right atrium through the coronary sinus. Other drainage patterns include draining into the left pulmonary vein or into the left atrium through its roof and sometimes through the coronary sinus when the defect exists between the coronary sinus and the left atrium. The aim of this report is to indicate the existence of PLSV in this environment and as one of the rarest abnormal venous connections limitedly reported in Nigeria. PLSV is a systemic venous drainage abnormality. Although it may be asymptomatic, it can also present with cardiac failure and severe life-threatening arrhythmias. Dilated coronary sinus during transthoracic echocardiography should be a pointer to a suspected PLSV.