Aims Periapical cysts of primary teeth are pathologic entities which are seldom encountered in the clinical practice. Most frequently, these lesions arise in correspondence with primary teeth presenting previous pulp therapy, severe carious lesions, or a history of previous trauma. The aim of the present study is to systematically review the treatment modalities of periapical cysts of the deciduous, along with the reporting of a clinical case. Materials and methods A case of periapical cyst treated with marsupialisationoccurring in an 11-year-old patient is described. A literature search was devised to retrieve studies reporting the treatment of periapical cysts, and involved papers published in the Cochrane Oral Health Group specialist trials, MEDLINE via PubMed, and EMBASE up to March 2023. A total of 39 articles were retrieved. Following title and abstract analysis, 27 articles were selected for full-text analysis, with the final inclusion of 24 articles. Results In total, 52 lesions were described in 50 subjects. Dental caries were more frequently associated with lesions' development, and primary molars were most frequently affected. The treatment approach was ether marsupialisation(8 studies) or enucleation (16 studies). Mean follow-up was 12 months. Recurrence of the lesions was not observed in any of the studies. Conclusion Periapical cysts of primary teeth present an overall good prognosis irrespective of the treatment option adopted. The performance of a prompt diagnosis appears of utmost importance, as the extraction of the primary teeth involved implies the management of the residual space for the correct positioning of the corresponding permanent teeth.