Cor triatriatum is a rare anomaly in which the left atrium is partitioned i
nto 2 distinct chambers with a variable degree of obstruction to left atria
l inflow. This anomaly often occurs in isolation but can occur in combinati
on with other cardiac lesions. The association of cor triatriatum. with hyp
oplastic left heart syndrome has not been reported. We describe a newborn w
ith hypoplastic left heart syndrome who also had the unusual finding of cor
triatriatum sinistrum.