The severity of seizures presenting to the emergency department ranges from benign to life threatening. There are also a wide number of possible etiologies. Computed tomography (CT) emergency imaging may be required at presentation to elucidate a possible cause and assess signs of intracranial trauma. This case describes a serious seizure episode in a young man while on holiday. A CT brain showed a skull fracture as a consequence of seizure-related head trauma but unexpectedly there were image findings consistent with encephalocraniocutaneous lipomatosis. The important radiological features of encephalocraniocutaneous lipomatosis and a differential diagnosis are presented.