Background: Pneumoceles of the paranasal sinuses are a very rare condition; characterized by a distended air-filled paranasal sinus extending beyond the margins of the paranasal bone, with bony defects and extension of air into the surrounding soft tissues. Also, spontaneous pneumo-cephalus is a rare condition which represented only 0.6% in the largest reported series of pneumocephalus. Although pneumocephalus caused by sinogenic origins, such as osteoma, has been reported, spontaneous pneumocephalus has not been reported as a complication associated with pneumocele of the frontal sinus. Methods: We report a case of spontaneous pneumocephalus associated with a pneumocele involving the frontal sinus which presented as acute severe headache. Results: To the best of our knowledge, this is the first case report in literature reporting spontaneous pneumocephalus in association with pneumocele of the frontal sinus. Conclusions: Although pneumocele of the frontal sinus is a very rare condition, it can develop into spontaneous pneumocephalus, and thus it needs to be included in the differential diagnosis of acute severe headache.