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Spontaneous lateral sphenoid cephalocele (SLSC) is the herniation of intracranial contents through a bony defect in the lateral sphenoid, without predisposing factors. SLSC pathogenesis is associated with idiopathic intracranial hypertension (IIH); however, the relationship between IIH and SLSC is not fully understood due to the limited number of published case reports. Here, we report a unique case of SLSC in a 39-year-old female who presented with a combination of a lateral sphenoid cephalocele and multiple radiologic findings indicative of IIH, some of which have never been described in previously published case reports. This case highlights the possibility that chronically elevated intracranial pressure may be a determining factor in SLSC development.