Choroidal melanoma is a relatively rare tumour with a poor prognosis, though it is the most common primary malignancy of the eye among adults. Choroidal melanoma has been reported to present as acute angle closure glaucoma, secondary glaucoma, chronic uveitis, cataract, and staphyloma. We report a case of a28-year-old male presenting with features of neovascular glaucoma in the right eye and having initially been treated with anti-glaucoma medications. However, ultrasonography revealed a mushroom-shaped, elevated, solid lesion with low to moderate internal reflectivity and regular internal structure suggestive of choroidal melanoma. Histopathological examination along with the immunohistochemistry studies of the lesion following enucleation of the eye confirmed the diagnosis of malignant choroidal melanoma. We highlight with this case that large choroidal melanoma may rarely present with features of neovascular glaucoma. The etiology of neovascular glaucoma should be investigated carefully and a potentially life-threatening intraocular tumour should be excluded, although it is a rare presentation.