Purpose: To report an unusual case of orbitocranial meningioma presenting in a neonate.

Methods: Case report

Results: A 1-day-old neonate was presented with severe proptosis of the left eye. CT scan and MRI showed an extensive mass with involvement of left orbit and brain. Discrete calcification could be seen on CT scan. The patient did not have any signs of neurofibromatosis and family history was negative. Histopathologic evaluation of orbital biopsy was compatible with meningioma.

Conclusion: This is an unusual presentation of meningioma in a neonate and to our knowledge this is the second report of such a case in the literature.