Background Intradiaphragmatic extralobar pulmonary sequestration(IDEPS) is a rare type of pulmonary sequestration (PS) and has been reported in very few studies. The purpose of this study is to access diagnosis and operative treatment of an IDEPS.Methods Patients with PS who were diagnosed and treated in our center from January 2015 to December 2020 were retrospectively analyzed, in order to identify patients with IDEPS. Clinical datas regarding diagnosis, surgical procedures and outcomes were documented.Results From January 2015 to December 2020, 215 patients with PS were surgically treated in our center, including 87 patients with extralobar pulmonary sequestration(EPS). 10 cases were identifed as IDEPS ( 2 males, 8 females; age, 5 months to 21 months). Prenatal ultrasounds and enhanced computed tomography (CT) showed the presence of IDEPS in 4 cases and 7 cases, respectively, and a three-dimensional (3D) reconstruction software was perfectly performed to identify the location of the lesions in 3 cases. The surgeries were smoothly performed by laparoscopic surgery in 1 case, video-assisted thoracic surgery (VATS) in 5 cases(Group 1) and Da Vinci robot-assisted thoracoscopic surgery(DVRATS) in 4 cases(Group 2). In Group 1 (5 females; the mean age, 8.0 months), the average operative duration, intraoperative blood loss volume, length of stay after operation, and postoperative thoracic catheter indwelling duration were 48 minutes, 3.8ml, 6.4 days and 2.2 days, respectively. That of Group 2(1 male, 3 females; the mean age, 9.5 months) were 80 minutes, 3.5ml, 4.3 days and 1.5 days, respectively. The IDEPS cases who followed up rang from 3 months to 36 months were included in this retrospective study and no side effect had appeared.Conclusions It’s challenging to diagnose an IDEPS through prenatal ultrasound or CT, and a 3D reconstruction software was proved to be capable to assist on the assessment of an IDEPS. We suggested early surgery to removel of an IDEPS, and the best path was accessing the mass from the chest. DVRATS or VATS for the treatment of an IDEPS is safe, feasible and effective. Furthermore, compared with VATS, DVRATS is 3D magnified view, more flexibility and precision.