Background Adult, benign, non-iatrogenic bronchoesophageal fistula (BEF) is a rare condition, which is occasionally described in single case reports. Therefore, little is known about its possible causes, presentation and management. Methods A systematic search of the literature in MEDLINE, PubMed Central and EMBASE databases between 1990 and 2020 was carried out to identify all cases of BEF. The initial database search identified 19,452 articles, of which 183 (251 individual patient cases) were included in the final analysis. Results Main causes of BEF were congenital malformations (97/251, 38.7%) and infections (82/251, 32.7%), while 33/251 (13.1%) fistulae were regarded as idiopathic and 39/251 (15.5%) attributed to other causes. Esophagograpy was the most sensitive method of diagnosis (97.4%) compared with esophagoscopy (78.9%), computed tomography (49.6%) and bronchoscopy (46.0%). Definitive treatment was surgical for 176 patients (70%), endoscopic for 25 (10%) and medical for 37 (14.7%). Compared with congenital BEFs, infective BEFs had shorter median symptom duration and were distributed more proximally over the bronchial tree. Definitive treatment was almost only surgical for congenital BEFs, while infective BEFs were treated also endoscopically (12%) and by medical therapy (38%). Morbidity, treatment failure and recurrence rates were higher for infective BEFs. Conclusions BEFs are rare. Symptoms are non-specific and a high index of suspicion is necessary for diagnosis. Patients with infective BEF tend to have a more severe clinical picture than those with congenital BEF. Surgery is the main treatment for patients affected by congenital BEF, while infective BEFs may heal conservatively.