Background:Intracranial arachnoid cysts are space-occupying lesions that typically remain stable or decrease in size over time. Cysts in infants younger than 1 year of age are remarkably different from those in older children and adults in terms of cyst localization and enlargement. Arachnoid cysts of the posterior fossa (PFACs) are very rare in infants and do not typically grow or present with clinical symptoms, such that surgical treatment is generally considered to be unnecessary. Here, we describe an extremely rare case of an infant with a rapidly enlarging symptomatic PFAC that was successfully treated with surgery.Case Description:A 4-month-old boy presented with increasing head circumference and a rapidly enlarging arachnoid cyst in the left posterior fossa with ventriculomegaly, which was documented using serial imaging over the preceding 2 months. We performed a microscopic resection of the cyst membrane to remove the mass effect as soon as possible and facilitate normal development. To confirm dural closure and prevent cerebrospinal fluid leakage, we also performed short-term (7 days) percutaneous long-tunneled external ventricle drainage after the surgery. Magnetic resonance imaging over a 4-year follow-up period revealed adequate reduction of the ventricle and cyst. The patient no longer exhibited progressive macrocrania and showed normal development.Conclusion:To our knowledge, this is the second successful case of surgical treatment of an enlarging symptomatic PFAC in an infant. Our surgical strategy for the treatment of this rare case can serve as a guide for surgeons in similar future cases.