Levonorgestrel-releasing intrauterine devices (LIUDs) are thought to release this progestin locally in the uterus to limit side effects. Authors here present a case of treatment-refractory hydrocephalus and pseudomeningocele (PMC), both of which fully resolved after LIUD removal.A 35-year-old woman with an implanted LIUD developed symptomatic PMC and hydrocephalus after suboccipital craniectomy for Chiari malformation type I. Over the next 8 months, she underwent ventriculoperitoneal shunt placement and two attempts at needle decompression of the fluid collection, which did not relieve her symptoms or the PMC, except for a few days at a time. Subsequently, she had her LIUD removed. Three weeks after removal of the LIUD, her symptoms as well as the fluid collection resolved completely without any further intervention. Thus, the increased intracranial pressure and associated persistence of the PMC may be partially attributed to the LIUD.This case indicates that a persistent problem (PMC and intracranial hypertension) that may be associated with the LIUD rapidly resolves after its removal. Implication of LIUDs as the cause of intracranial hypertension is still a matter of controversy. Further studies are needed to evaluate any potential causal relationship between LIUDs and intracranial hypertension, and physicians are advised to consider this scenario in their differential diagnosis.