Background: Tetralogy of Fallot (TOF) is the most common combined the deformity of unilateral absence of the pulmonary artery (UAPA). The treatment strategy of TOF combined with UAPA is still very controversial. In this study, we analyzed the effect of the bilateral pulmonary reconstruction for patients with TOF combined with UAPA. Methods: A single-institution, retrospective review of all 1713 patients with TOF between January 2009 to November 2021. Eight patients were diagnosed with TOF combined with UAPA, and 7 patients underwent the surgery. Three patients underwent one-stage TOF correction with bilateral pulmonary artery reconstruction. Three patients underwent bilateral pulmonary artery reconstruction, followed by two-stage TOF correction after several months. One patient underwent 2 procedures of left pulmonary artery reconstruction, and the VSD remained open. Results: All 7 patients survived during the postoperative follow-up and showed good cardiac function and normal oxygen saturation >97%. During the follow-up with echocardiograms, it was found that the left pulmonary arteries that were reconstructed with Goretex vessel or direct anastomosis had thrombosis or stenosis. The left pulmonary artery reconstructed with the modified autologous tissue extension technique was unobstructed. Conclusions: In patients with TOF and UAPA, if there is a pulmonary confluence in the affected hilum, it is feasible to implement bilateral pulmonary artery reconstruction for one-stage TOF correction. The use of pulmonary artery extension technique and autologous tissue to bilateral pulmonary reconstruct could reduce the incidence of anastomotic stenosis.