Abstract Purpose Few studies address the surgical correction of pectus excavatum (PE) in patients with connective tissue disease (CTD). We have identified the preoperative characteristics, postoperative complications, and outcomes of patients with CTD undergoing bar repair of PE and compared these outcomes to a control group without CTD. Methods A retrospective review of patients undergoing primary repair of PE with a bar procedure from 1997 to 2006 identified 22 patients with CTD. Of those, 20 (90.9%) had their bars removed. We identified 223 patients of similar age without CTD whose bars were removed. Data collected included demographics, preoperative symptoms, operative characteristics, and postoperative outcomes. Results Among those with CTD, the median age at repair was 15.5 years, with a mean pectus index of 4.0 ± 1.4. Three patients (13.6%) experienced bar displacement or upper sternal depression requiring surgical revision. Only 1 patient recurred after bar removal. Rates of bar displacement, upper sternal depression, and recurrence were not statistically different than those in the comparison group. Conclusions Patients with CTD benefit from primary bar repair of PE and experience excellent operative outcomes after repair, with complication rates being no different than those found in similarly aged control patients.