The retinal astrocytic hamartoma is a hallmark ophthalmic feature of tuberous sclerosis complex (TSC).1 The typical course of such tumours is a relatively slow-growing nonaggressive lesion in the retina that can be managed expectantly.2 Nonetheless, there have been several reports in the literature of aggressive astrocytic hamartomas that do not demonstrate this benign course.3 We present here a rare case of an aggressive tumour in a patient with TSC that has cell types more typical of the subependymal giant cell astrocytoma (SEGA) that is seen in the brains of such patients.