A 26-year-old man presented with an insidious loss of vision OD. He had amblyopia OD as a child secondary to congenital esotropia. Ophthalmic examination revealed a large mass beneath his right retina near the posterior pole. He also had glaucoma and a rapidly developing cataract. Ultrasound detected a choroidal mass, which was consistent with a melanoma. The patient chose enucleation over radiation therapy as his treatment modality. Pathologic evaluation of the tumor revealed a necrotic melanocytoma with a large malignant melanoma arising from its center that was comprised mainly of epithelioid cells. Malignant melanoma arising in a necrotic melanocytoma is an extremely rare event, and we believe this to be the first description of this event in a young patient with an amblyopic eye.