Exploring Aneurysmal Bone Cysts of the Skull: Insights from a Review of the Literature and a Case Report.

: Aneurysmal bone cysts (ABCs) are rare bone tumors that can occur in the skull, leading to extensive bone destruction and compression of surrounding tissues. Due to the rarity of these lesions, there are limited data available in the literature, which primarily consists of case reports. We aimed to collect and analyze the available data to summarize the current state of knowledge on this rare pathology, while also conducting a statistical analysis to identify potential risk factors and management strategies. : A review was performed following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, covering studies published from January 1950 to December 2023. A total of 60 articles and 74 case reports were included. : The mean age at diagnosis was 14.8 ± 12.5 years, with slightly higher male gender predominance. Regarding the different skull bones, a statistically significant higher growth trend of ABCs was found at the parietal bone in the male population ( = 0.025). At the occipital bone, a significant correlation was observed with the age of incidence for symptomatic lesions ( = 0.007) and development from fibrous dysplasia ( = 0.019). Secondary lesions showed a higher frequency of complications within the first months post-surgery ( = 0.041). : No significant correlation was found between ABCs and fibrous dysplasia (FD) or head trauma. Male patients with FD showed a higher tendency to develop an aneurysmal cyst at the occipital bone at an older age and a higher tendency for growth in ABCs at the parietal bone. However, to date, no molecular or genetic correlation with male hormones has been reported in the literature. Surgery remains the only effective treatment, but complications should be carefully considered, particularly in patients with pre-existing pathological conditions.