<p>Neuroborreliosis is a well-described complication of Lyme disease in the pediatric population. Intracranial hypertension can occur with this condition in children presenting with transient or permanent vision loss in severe cases. A systematic review of the literature was undertaken to answer the following questions: what forms of neurosurgical management have been used in the treatment of intracranial hypertension in pediatric Lyme disease, what are the indications for such neurosurgical management, and what outcomes are associated with these interventions? 43 publications were identified including 131 cases of intracranial hypertension secondary to neuroborreliosis. Of these, 4 cases were identified where neurosurgical intervention was required for the management of intracranial hypertension in neuroborreliosis. All cases involved cerebrospinal fluid diversion procedures including lumbar drains, ventriculoperitoneal shunts, and external ventricular drains. Neurosurgical intervention was utilized for intracranial hypertension refractory to medical therapy in all cases, with all patients experiencing vision loss. Resolution of papilledema was observed in all cases. 2 of 4 cases experienced complete resolution of vision loss, while 2 had long lasting visual deficits despite intervention. This review highlights the role for cerebrospinal fluid diversion in refractory intracranial hypertension secondary to neuroborreliosis. Timely recognition, awareness of this potential complication, and early neurosurgical involvement, if necessary, may help improve outcomes for this patient population. Further study is warranted.</p>