Introduction: Aneurysmal bone cysts (ABC) are pseudocysts expansive lesions. The World Health Organization (WHO) categorizes primary ABCs as cystic neoplasms that contain fibrous septa separating numerous blood-filled spaces. Approximately 70% of ABC cases are primary, while 30% are secondary. Objective: The present study aimed to carry out a concise systematic review of the main clinical findings of aneurysmal bone cysts, highlighting the incidence and prevalence, as well as the main forms of treatment. Methods: The rules of the Systematic Review-PRISMA Platform were followed. The research was carried out from May 2022 to July 2022 and developed based on Scopus, PubMed, Science Direct, Scielo, and Google Scholar. The quality of the studies was based on the GRADE instrument and the risk of bias was analyzed according to the Cochrane instrument. Results: A total of 143 articles were found. In total, 73 articles were evaluated and 10 were included and evaluated in the systematic review. The symmetric funnel plot does not suggest a risk of bias. After prospecting the main literary findings, it was generally evidenced that the aneurysmal bone cyst (ABC) is a rare lesion that is more frequently found in young adults and children. It may have unpredictable behavior, with a high recurrence rate after treatment. Standard treatment consists of curettage plus local adjuvants and bone grafting to fill the void. In anatomic sites with difficult surgical access, percutaneous procedures are used (injection of sclerosing agents, radiofrequency thermal ablation, or selective arterial embolization. Medical management with bisphosphonates or denosumab has also been advocated. Conclusion: Primary ABCs involving the maxillofacial region are extremely rare and even more in patients with advanced age. Next-generation sequencing confirmed the presence of a USP6-CDH11 fusion gene, consistent with classification as a primary aneurysmal bone cyst. Sclerotherapy provided an effective and minimally invasive treatment for aneurysmal bone cysts and is particularly useful for deep injuries, challenging surgical access, and potentially injurious vital structures.