An 11-year-old girl with a history of migraine with aura later developed episodes of loss of consciousness with tonic–clonic seizures. Headache attacks presented initially with a mild severity and responsiveness to acute treatment, then progressively worsened both in frequency and severity, and also did not respond to prophylactic treatment with flunarizine. In two distinct episodes of her typical migraine with aura, she experienced seizures within 20 min. At the EEG (electroencephalography), epileptic graphic elements, with sharp waves in the right centrotemporal region, were revealed, while the brain MRI (magnetic resonance imaging) was unremarkable. Given the temporal relationship between her aura and seizures, she was diagnosed with migraine aura-triggered seizures. Antiepileptic therapy with topiramate was administered, leading to seizure and migraine control. This case highlights the diagnostic challenges of distinguishing migraine aura-triggered seizures from occipital epilepsy and other mimics. It underscores the importance of careful differential diagnosis and the potential role of antiseizure drugs in managing this rare condition, which sits at the intersection of migraine and epilepsy.