Symptomatic cerebral edema from diabetic ketoacidosis occurs infrequently but carries a high rate of mortality and morbidity owing to complications from intracranial hypertension. Treatment options are limited but include hyperosmolar therapy with mannitol or hypertonic saline, tracheal intubation for airway protection, and hyperventilation via mechanical ventilation. We describe here the successful use of an intracranial pressure/cerebral perfusion pressure-targeted management strategy through ventriculostomy catheter placement with intracranial pressure monitoring and cerebrospinal fluid drainage, hyperosmolar therapy with hypertonic saline, and controlled hyperventilation to treat life-threatening complications of cerebral edema in a pediatric patient with severe diabetic ketoacidosis.