INTRODUCTION: Bronchopulmonary sequestration is a rare congenital pulmonary malformation. A sequestration is generally defined as non-functioning lung tissue that is not in normal continuity with the tracheobronchial tree and derives its blood supply from systemic vessels. OBJECTIVE: To define the main clinical and radiologic features of intra- and extralobar sequestration of the lung, to study vessel variability and to analyse case of not obvious CT images of intralobar sequestration that was diagnosed intraoperatively. MATERIALS AND METHODS: We analysed literature found in PubMed and ScienceDirect regarding lung sequestration since 1979. The case report of a patient with extralobar sequestration diagnosed intraoperatively.RESULTS: The main clinical and radiologic features of lung sequestration, challenges of differential diagnosis were summarised, including specificity of case report.DISCUSSION: Presence of aberrant vessel requires verification of pathology before operation due to high risk of haemorrhage. The leading role in the verification belongs to radiologic imaging, however in rare cases aberrant vessel may not be visualised due to small diameter or occlusion.CONCLUSION: The anatomy of aberrant vessels in sequestrations is extremely variable. In case report sequestration was seen in an adult. The pathology was not diagnosed prior to the operation due to failure of the vessel visualisation. Knowledge of possible diagnostic challenges decreases the risk of intraoperative complications.