Objective/Hypothesis To examine the clinical features of benign intratracheal thyroid (ITT) and their management strategies and outcomes. Study Design Case series study. Methods This systemic review was conducted in two international academic centers. This review includes 43 patients: one new case from the Massachusetts Eye and Ear Infirmary, four new cases from Beijing Tongren Hospital, and 38 previously published cases. We analyzed these 43 cases and summarized the patients' epidemiological data, clinical features, and treatment regimens. Results ITTs were less common in men than in women (male:female ratio of 3:10). ITT was observed in patients as young as neonates and as old as 85 years. Orthotopic thyroid nodules were present in 55.8% of the patients with ITT. Malignancy was incidentally found in 4.6% of all ITTs. Imaging examinations showed that the ITTs were typically attached to the posterolateral/lateral tracheal wall of the first, second, or third tracheal rings. Tissue attachment between the ITT and normal thyroid lobes was seen in 59.5% of the patients. Thirty-seven patients underwent surgery: 30 underwent open surgery, and seven underwent endoscopic debulking resections. One neonate received thyroid suppression therapy. One patient with ITT and papillary thyroid cancer was treated with radiotherapy and ultimately died after recurrence. Conclusions Surgical resection is an effective treatment for benign ITT. We hypothesized that abnormalities during the embryonic development of Berry's ligament might play a role in ITT pathogenesis. Level of Evidence NA Laryngoscope, 2021