Background: Styloidogenic jugular venous compression syndrome (SJVCS) has been shown to present with a similar symptomatology to idiopathic intracranial hypertension (IIH) and is caused by compression of the internal jugular vein (IJV) between the lateral tubercle of C1 and the styloid process. Treatments including venous stenting and styloidectomy have been reported with good outcomes; however, treatment of a pediatric patient with SJVCS with styloidectomy has not previously been reported in the literature. Case Report: A 12-year-old male presented with refractory positional headaches, nausea, and vomiting, and after, workup including lumbar puncture (LP) and intracranial pressure (ICP) monitoring was found to have intracranial hypertension associated with contralateral neck turning. Computed tomography venogram (CTV) revealed severe bilateral compression of the IJV’s between the styloid processes and C1 tubercle. The patient was successfully treated with unilateral right-sided styloidectomy with symptomatic relief. Conclusions: This is the first reported pediatric case of SJVCS treated successfully with styloidectomy to our knowledge and adds to the limited literature that styloidectomy is a durable treatment option for SJVCS.