Abstract
Primary orbital melanoma is rare and has varied initial presentation. A 28-year-old female presented with proptosis and decreased vision in the left eye. Computed tomography scan showed an orbital mass with contrast enhancement and calcification around the optic nerve leading to a diagnosis of meningioma. The patient chose to be on observation. Loss of vision with an increase in proptosis was seen at 6 months follow-up. On surgical exploration, a well-defined pigmented mass was seen encasing the optic nerve. Histopathological analysis revealed a malignant melanoma. Metastatic workup was negative. Left eye lid sparing exenteration was done. A high index of suspicion is necessary in a rapidly growing suspected optic nerve sheath meningioma and a differential diagnosis including orbital melanoma be considered.