Pineal cysts are usually benign, fluid-filled sacs and most pineal cysts are asymptomatic. Sudden death specifically related to pineal cysts is extremely uncommon. A literature review of the pertinent databases, including PubMed, Google Scholar, Scopus, and Web of Science, was carried out to review the existing literature describing sudden death in patients with pineal cysts. In the evaluation of 49 articles, it was found that four reports discussed the unexpected death of patients who had pineal cysts. A total of four cases of sudden death and a pineal cyst were reported. There were 75% females and a mean age of 29 (range: 20-45). Cyst size on average was 1.3 cm (1.2-1.5). In each case, the cause of death and the involvement of important brain structures were confirmed by autopsy results. A pathological analysis of the pineal region and the surrounding brain tissue revealed a variety of lesions. Vascular malformation was found in one case, adding another layer of complexity to the study of sudden death syndrome. In this research, the authors highlight the fact that patients with pineal cysts can experience serious, even fatal, complications. Increased vigilance and early detection through neuroimaging and neurological assessments are required due to the wide variety of clinical manifestations and underlying mechanisms. To explain the mechanism and enhance the management and prevention of sudden deaths associated with pineal cysts, additional research with larger sample sizes is required.