This article presents a review of the literature on congenital bilio-bronchial fistula (BBF), a rare anomaly characterized by abnormal communication between the bile duct and respiratory tract. Congenital BBF often presents with bilioptysis in early neonates and infants; however, patients with no overt symptoms may occasionally present in adulthood. Our literature search in Medline from 1850 to 2023 revealed 42 reported cases of congenital BBF, primarily managed with thoracotomy and excision of the fistula tract. About one-third of these cases required multiple surgeries due to associated biliary anomalies. The review underscores the importance of diagnostic imaging, including bronchoscopy, in identifying and delineating the extent of the fistula. It also highlights the evolving surgical management, with recent cases showing the efficacy of minimally invasive approaches such as laparoscopy and thoracoscopy. In addition to the literature review, we report a young female patient with a history of recurrent respiratory infections presenting with bilioptysis and extensive left lung damage. Initial management included bronchoscopy-guided glue instillation, left thoracotomy, and pneumonectomy. Following the recurrence of symptoms, the patient was successfully treated with laparoscopic excision of the fistula tract. In recent times, minimally invasive approaches such as laparoscopy and thoracoscopy, with excision of the fistula tract are gaining popularity and have shown good results. We suggest biliary communication being the high-pressure end, tackling it transabdominal may prevent recurrent problems.