An 11-year old boy presented to his optometrist with a 2-week history of intermittent flashing lights and blurred vision in his right eye. His medical background included amblyopia secondary to strabismus and a port-wine stain (naevus flammeus) above his right eye, both of which were corrected in early childhood with patching and laser treatment, respectively. Whilst at the optometrist, fundoscopy was performed which highlighted an ambiguous lesion in his right eye temporal to the optic disc that could not be characterised. He was referred urgently to the acute eye clinic, where a B-scan and fundus fluorescein angiography (FFA) were performed. These investigations, along with slit lamp examination, were highly suggestive of a choroidal haemangioma. Given his history of a port-wine stain, he was referred to a specialist centre to explore the possibility of a diagnosis of Sturge-Weber syndrome. This article aims to shed light on this rare neurocutaneous disorder, and the ocular manifestations associated with these affected individuals.