Introduction:

Osteogenesis imperfecta is a group of heterogenous abnormalities of the collagen synthesis, characterized by multiple fractures and deformities. Thoracic surgery in patients with osteogenesis imperfecta is not common.

Clinical case:

We present a case of a 30 years old male with a previous diagnosis of osteogenesis imperfecta who was referred to our center for presenting a loculated empyema on his right hemithorax. A right posterolateral thoracotomy was performed. In the middle of the surgery we visualized an anatomical abnormality that coincides with an accessory fissure of the superior lobe.

Conclusion:

Osteogenesis imperfecta is a rare clinical entity, however the surgeon must take into consideration the physiopathological implications during the surgery.