Figure 1: (a) Clinical photograph of the patient at presentation showing abaxial proptosis with supero-temporal dystopia (b) clinical photograph at 9 months follow-up post six cycles of chemotherapy showing with almost complete symptomatic improvement except for slight globe displacement and no signs of recurrence (c) axial and coronal sections (d) of contrast-enhanced MRI orbit with brain showing an ill-defined multilobulated mass, measuring 3.6 × 3.1 cm with intense homogenous enhancement on both T1 and T2 images in the right retrobulbar space involving the right ethmoid sinus (e) coronal section of whole-body PET-CT scan showing a large FDG avid lesion in the right orbit involving the right ethmoid sinus, with no intracranial extension (f) Syncytial arrangement of small round tumor cells with finely granular chromatin. (HandE ×200). (g) the tumor cells are surrounded by a richvascular network. Frequent mitotic figures are seen (arrow) (HandE ×200)(h) tumor cells demonstrate strong cytoplasmic staining for cytokeratin AE1/AE3 and synaptophysin, and (i) Avidin Biotin ×400