To investigate how different presentations of posterior fossa arachnoid cysts (PFACs) influence surgical indication and strategies and their impact on clinical and radiological outcome in children, proposing a treatment flow-chart. In this retrospective study, children < 14 years old with PFAC diagnosed at IRCCS Giannina Gaslini Hospital from 2008 to 2023 were identified Patients showing a mega cisterna magna (MCM), Dandy-Walker syndrome (DWS), Blake's pouch cyst (BPC), neuroenteric cysts or multiple cysts were excluded. Data regarding type of treatment, age at surgery, surgical complications were collected and analyzed. Clinical and radiological outcomes were considered at 1-, 3- and 5-years follow-up. A "wait and see" strategy in asymptomatic showed better clinical outcomes at 1-year follow-up (p = 0.047). No significant difference in clinical outcome or risk of re-surgery were observed between microsurgical fenestration, endoscopical fenestration or shunting were reported, while location of the cyst influenced surgical strategy (p = 0.015). Age < 12 months at surgery (p = 0.008), hydrocephalus (p = 0.001), especially when associated with macrocephaly (p = 0.004), and the placement of a shunt (p = 0.006) resulted as risk factors of re-surgery. An association between radiological and clinical outcomes was observed at 1-year follow-up. Treatment decision should be based on clinical presentation rather than radiological presentation. While a "wait and see" strategy is suggested in asymptomatic patients, when surgery is indicated, several factors should be considered, such as presence of hydrocephalus, location of the cyst and age of the patient, to improve clinical outcomes, reducing complications.