Congenital absence of unilateral pulmonary artery, either as a primary defect or in combination with other congenital cardiovascular malformations, is very rare. Double outlet right ventricle pathology in combination with unilateral absence of pulmonary artery has only been reported once in the literature. In this report, we present our experience with double outlet right ventricle with unilateral absence of left pulmonary artery in a 3-year-old female patient who underwent a palliative procedure and was scheduled for correction.