Background Esophageal duplication cysts are a rare congenital cystic malformation from faulty intrauterine recanalization of the esophagus during the 4-8 th weeks of development. They account for 20% of all gastrointestinal duplication cysts and commonly involve the distal esophagus. Presenting symptoms may be related to size and location. Materials and Methods Following the PRISMA guidelines, a systematic review was performed by searching published literature in various databases. Data from 97 reported case reports were pooled to present a descriptive and statistical analysis. Results Patient population was composed of 51(52.5%) males and 46 (47.5%) females, and mean ages was 42.3 years (18-77). Distal cysts were the most prevalent. Seventy-nine (81.4%) patients were symptomatic; common symptoms included dysphagia, chest pain, cough and weight loss. Fifteen (15.5%) patients were treated conservatively and 75 (84.5%) by surgical treatment, among them thoracotomy in 30 (30.9%) patients and VATS in 17 (17.5%) patients. Mean length of hospital stay was 8.6 days (range: 1-26 days). One fatality was registered. Location, unlike size, was not found to influence presenting symptoms or treatment employed. Frequency of conservative treatment was not significantly different between symptomatic and asymptomatic patients. Open approaches were associated with longer stays than their minimally invasive counterparts. Conclusion Esophageal duplication cysts remain rare in adults and are frequently located in the distal esophagus. Larger cysts are more likely to cause symptoms. Various surgical techniques may successfully be employed in the treatment of this pathology. Minimally invasive procedures have a shorter hospital stay.