Abstract
Neuroblastoma (NB) is a malignant neoplasm accounting for 7.5% of malignancy in children. It can originate anywhere along the sympathetic chain with the adrenal medulla being the most common site in 35% of cases. The initial presentation of orbital metastasis is very unusual that accounts for only 8% of all NB cases. We report a rare case of a 16-month-old girl who initially presented with bilateral rapidly progressive ocular proptosis for two weeks. CT scan of the brain and orbits revealed bilateral heterogeneous lateral orbital lesions, and CT scan of the abdomen and pelvis revealed huge heterogeneous right adrenal lesions. Histopathology of the abdominal mass confirmed the diagnosis of stage IV NB with orbital metastasis and the patient was started on an aggressive chemotherapy regimen. Ophthalmologists have a vital role in the diagnosis of NB which should be considered in the differential diagnosis of any rapidly progressive proptosis in young children. Early investigation and systemic workup should be made immediately, as it is a potentially life-threatening malignant tumor that requires aggressive management.