Umbilical cord cysts (UCCs)—cysts located in the umbilical cord that are derived from an abnormal embryonic development process—are typically an incidental discovery during prenatal ultrasound. It can be described as either a pseudocyst or a true UCC, which results from focal edema or degeneration of Wharton jelly or the remnants of embryonic development, respectively. Due to the relative rarity of the UCC, the clinical guidance of UCCs is not yet available. Herein, the aim of this paper is to discuss the classification, diagnosis, prognosis, and clinical management of UCCs through a literature review, in order to improve the understanding of UCCs among clinical obstetricians and pediatricians.