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Neurosurgical diseases in pregnancy simultaneously jeopardize two lives and represent both medical and ethical problem. Upon confirming the presence of intracranial malignancy in pregnancy, further procedure is very individual and it implies cooperation of gynaecologists, neurologists, neurosurgeons, oncologists, anaesthesiologists and neonatologists.

Introduction. Occipitocervical (OC) fusion is a method for fixation of the OC junction when there is instability of that segment. Arnold Chiari malformation is a congenital disorder where cerebellar tonsils descend through the foramen magnum, which can lead to cervicomedular compression and formation of syrinx. While treating this condition, for the purpose of decompression, the foramen magnum is expanded which can potentially harm the stability of the OC junction. Case outline. We are presenting the case of a 16 year old female who was surgically treated (suboccipital craniectomy and decompression) because of Arnold Chiari malformation type I. One-year post-op multislice computed tomography verified a slight enlargement of basion-axial and basion-dens intervals, with signs of spinal cord compression. Surgery was performed - occipitocervical fusion, canal decompression on C1 and C2 levels with a plate on occipital bone and screws placed in the third, fourth, and fifth cervical vertebra. Conclusion. Occipitocervical fusion is a complex surgical procedure (vital neurovascular structures), but it is a reliable method for treatment of instability of the OC junction.