BACKGROUND/PURPOSE: Intradiaphragmatic extralobar pulmonary sequestrations (IDEPSs) are a rare subset of bronchopulmonary sequestrations (BPS). We report the largest series of patients with IDEPS and describe the diagnostic and operative challenges associated with this condition. METHODS: We retrospectively reviewed our experience with fetal and pediatric BPS from 1995 to 2010 to identify patients with IDEPS. RESULTS: We identified 27 patients with BPS and 4 patients in whom the masses were within the diaphragm. In 1 patient, the prenatal ultrasound correctly identified the mass as being within the diaphragm itself, whereas the remaining cases were thought to be intraabdominal or had discordant preoperative imaging findings. The diagnosis of an IDEPS proved challenging to make prospectively using prenatal ultrasound, computed tomography, or magnetic resonance imaging. All patients underwent attempted resection. Two cases required a combined laparoscopic and thoracoscopic approach to accurately localize the mass. The postoperative recovery of these patients was uneventful. CONCLUSIONS: We present the largest reported experience of IDEPS. Because preoperative imaging studies cannot always determine whether a sequestration is intraabdominal, intrathoracic, or intradiaphragmatic, operative planning may pose a challenge. However, the use of minimally invasive approaches can allow exploration of both the thoracic and abdominal cavities with low morbidity.