Pneumocephalus is commonly associated with head and facial trauma, ear infection or surgical interventions. We describe the rare case of a spontaneous pneumocephalus arising from lateral mastoid air cells. A 48-year-old man presented with a 10-day history of sudden, repetitive, 'hammering-like' acoustic sensations in his left ear that were followed by word-finding difficulties and loss of vision in the right visual field. Imaging revealed a large, left temporal pneumatocele associated with a small acute intracerebral hemorrhage. Left temporal and subtemporal craniotomy and decompression were performed. Further exploration confirmed a dural and osseous defect in the anterolateral surface of the mastoid that was consecutively closed watertight. Although extremely rare, a spontaneous pneumocephalus with mastoidal origin should be considered as a possible diagnosis in patients with suggestive acoustic phenomena and other non-specific neurological symptoms.