The association of hypoplastic left heart syndrome with valvular pulmonary stenosis is rare and is not well-described in the literature. Here we describe the experience of Norwood stage one reconstruction in an infant with hypoplastic left heart syndrome and significant pulmonary stenosis. The baby was successful palliated and stage II reconstruction with a bidirectional Glenn shunt was also later performed. Even though this condition is considered a poor candidate for Norwood stage one reconstruction because of outflow obstruction, Norwood stage one reconstruction was used because neonatal transplantation is not a feasible option in Taiwan. This experience suggested that Norwood staged reconstruction is an option for this difficult condition.