Objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) occurs in patients with recurrent or chronic pulmonary embolism, and is a rare but potentially devastating disease in adolescents and young adults. Pulmonary thromboendarterectomy (PTE) is an important curative therapy for patients with CTEPH. The importance of this treatment may be under-appreciated and under-utilized in adolescents and young adults. Study Design: We performeda chart reviewof all patients <24 years of age who had PTE performed for CTEPH at our institution from 2003-2009 (seven patients, average age 20.2±2.5 years, range 17-23 years, average BMI 34±12 kg/m2). All patients had their operations performed by a single surgeon. Results: There were no deaths. Three of the seven patients were incorrectly diagnosed prior to the diagnosis of CTEPH. Right ventricular systolic pressure decreased from 61.3±19 mmHg preoperatively to 31.8±5mmHg post-operatively (P=0.008). All patients reported symptomatic relief, and no patient has had recurrence of CTEPH. Conclusion: Although rare in the young patient, recurrent pulmonary emboli can have devastating consequences. The potentially lethal implications of CTEPH may be under-appreciated in adolescents and young adults. When indicated, PTE is a definitive, curative procedure with low morbidity and mortality. © 2010 Wiley-Liss, Inc.