We are reporting an unusual case of reactive lymphoid hyperplasia of choroid. The ancillary laboratory parameters, intraocular fluid analysis and whole body imaging scans ruled out any infectious etiology or any extraocular foci of primary malignancy. Due to no useful vision in the affected eye and increasing size of the lesion, the eye had to be enucleated. Histopathology, immunophenotyping and molecular study of the enucleated globe confirmed the diagnosis of reactive lymphoid hyperplasia of choroid.