We present a case of tuberous sclerosis, classified as phacomatosis. The patient is a young woman admitted to hospital for a tumor in the right eye. The diagnosis of tuberous sclerosis was based on the presence of the various symptoms of this disease. The patient's ophthalmological symptoms were severe. Ophthalmologists must look for these symptoms in all subjects at risk, particularly in patients with epilepsy but also in the presence of cutaneous or visceral symptoms.