Because the outcome of a large clinical series of patients with juxtaposition of the atrial appendages (JAA) has not previously been reported, a retrospective study was performed on patients diagnosed with JAA at a tertiary medical center. Patients with JAA were identified through a computerized database search, and echocardiograms and medical records of patients with JAA were reviewed. Twenty-two patients with JAA were identified, with an overall incidence of 0.28%. All but 2 patients were diagnosed prospectively with JAA by echocardiography. The lesion-specific incidences and associated lesions were similar to those of large autopsy and surgical series. Abnormal conotruncal anatomy was more frequently seen with juxtaposition of the right atrial appendage (JRAA) vs juxtaposition of the left atrial appendage (JLAA) (14/15 vs 4/7), as was atrial outlet obstruction (6/15 vs 2/7). JLAA was more frequently associated with complex atrioventricular anatomy (3/7 vs 1/15). Patients with JAA underwent single ventricle palliation in 11/22 cases with 6 deaths; biventricular repair was performed in 8/22 cases with no deaths. Surgical outcomes for patients with JRAA and JLAA were similar, and survival was predominantly influenced by suitability for biventricular repair.