Cor triatriatum is a rare congenital heart malformation with different natural history which depends on the size of the aperture in the partition between the proximal and distal chambers. In classic cor triatriatum all pulmonary veins return to proximal chamber (accessory left atrium, aLA), but this case we reported with left upper pulmonary vein returned to distal chamber (true left atrium, tLA). The partition had one 5mm aperture that allowed this patient grown up to adulthood without any symptoms, but finally resorted to hospital with symptomatic atrial flutter and detected this cor triatriatum accidentally. Atrial flutter may be a long-term complication of cor triatriatum during its natural history, surgical correction of cor triatriatum and radiofrequency ablation for atrial flutter is recommended once diagnosed.