We report a rare case of a bilaterally blind, 33-year-old male, who presented with rapidly progressive, painless swelling in the right orbit for two months, in the absence of ocular trauma. Suspecting an intraocular neoplasm, a contrast-enhanced MRI (CE-MRI) orbit was performed, which was suggestive of a right-sided superomedial eccentric soft-tissue lesion with bilateral rudimentary globes associated with cyst, hypoplastic optic nerves, and focal areas of calcification. Diffusion-weighted MRI demonstrated diffusion restriction and yielded an indeterminate value of apparent diffusion coefficient (ADC). A right enucleation with excision of the cyst was performed. Histopathological examination confirmed the diagnosis of a right-sided massive retinal gliosis (MRG) with bilateral microphthalmia with cyst. This case demonstrates the role of a detailed histopathological analysis along with immunohistochemistry (IHC) in differentiating MRG from a neoplasm.