BACKGROUND: Cervical endometriosis (CE) is an uncommon diagnosis with a paucity of evidence on its management. OBJECTIVE: To examine 10 years of data to identify CE cases and to conduct a systematic review to evaluate evidence for managing CE. SEARCH STRATEGY: Cases of CE were obtained retrospectively via a pathology coding system. Literature search was performed using Ovid MEDLINE, EMBASE, and Cochrane Library (1949-2022). SELECTION CRITERIA: Studies on diagnoses and management of CE were included, and those with retrospective diagnosis of CE on histology and no further management were excluded. DATA COLLECTION AND ANALYSIS: An Excel spreadsheet was used for data collection. Two independent authors went through individual cases, extracting and analyzing data. MAIN RESULTS: In case series, women presented with intermenstrual bleeding (IMB)/post-coital bleeding (PCB) n-25, (30%), followed by abnormal uterine bleeding (AUB) n-24, (29%), dysmenorrhea n-12, 12%, and/or dyspareunia (12%). In all, 44% had an initial cervical biopsy confirming CE diagnosis and 48% had CE on hysterectomy specimen. Main reasons for hysterectomy were AUB (36%) and fibroids/adenomyosis (28%). In the systematic review, 1737 studies were identified from databases, 181 were assessed for eligibility, and 14 studies discussed individual case reports and case series specifically on treating CE. Nine full-text and abstract studies were available to identify potential treatment options for CE. Treatments were based predominantly on presentation at time of diagnosis. CONCLUSION: Findings from case series suggest that CE was the main diagnosis in women who had a hysterectomy for AUB or had a cervical biopsy for PCB/IMB. Investigations should be considered to rule out CE to have the potential for conservative alternative treatment rather than major surgery. There is no clear guidance on managing CE and good-quality studies are required to provide conclusive treatment options.