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Transient cortical blindness is a rare and dramatic complication after coronary angiocardiography in adulthood. This potential complication of transcatheter procedures which are widely used in the diagnosis and treatment of congential heart disease has not been reported during childhood. Although the exact mechanism is not fully understood, the direct idiosyncratic neurotoxicity is the most reasonable explanation. In this manuscript, we present a nine-year-old child who developed transient cortical blindness following diagnostic angiography with vertebro- venous fistula (VVF) which might be the cause of this complication. In regard to this case, the pathogenetic mechanisms and management of this rare complication were also discussed.