A 29-year-old male known case of vernal keratoconjunctivitis (VKC) presented with 5-month history of a rapidly growing mass on the temporal aspect of the right cornea. The 9 × 9 mm epibulbar lesion was excised and histologically showed features of extranodal Rosai Dorfman disease (RDD) with emperipolesis of eosinophils. The lesion did not recur following excision. The association of RDD with VKC has not been previously reported; however, the causal relationship remains unclear.