Cranial extradural haematoma (EDH) is a neurosurgical emergency that can be caused by traumatic or non-traumatic causes with the former being more prevalent. Non-traumatic causes are variable and can include infection, vascular malformation and haematological disorders. This paper will address an extremely rare non-traumatic cause of EDH. More specifically, eosinophilic granuloma (EG), the localized form of Langerhans histiocytosis, may involve the skull and has rarely been reported to present with EDH. The case that will be presented is that of a three-year-old male patient, who presented with progressive vomiting and drowsiness, associated with left parietal swelling. CT scan of the brain showed an extradural haematoma and an osteolytic parietal lesion. He underwent emergent craniectomy, evacuation of the haematoma and dura resection as the lesion was infiltrating the dura. Histopathological examination of the dura and the bone edges showed eosinophilic granuloma (EG). The mechanism of a haemorrhage in this situation is poorly understood and the literature is extremely scarce. In conducting a thorough literature review, only 11 case reports of EG causing non-traumatic EDH were found. The details of these 11 cases will be reviewed and discussed in this paper, in addition to our illustrative case.