Introduction: Unicornuate uterus is a rare Müllerian anomaly. Its potential association with a rudimentary uterine horn can cause a diagnostic delay. The most common consequences are pelvic pain, hematometra and endometriosis. Diagnosis of a unicornuate uterus is usually done by imaging combining ultrasound (US) and Magnetic Resonance Imaging (MRI). The aim of this systematic review is to assess the role of laparoscopic approach in the management of this rare condition. Methods: A comprehensive search was performed in PubMed, EMBASE, SCOPUS and Web of Science databases prior to 1 may, 2022 according to Preferred Reporting Items for Systematic Reviews and Meta- Analyses statement (PRISMA). The inclusion criteria were: cases of rudimentary horn managed through laparoscopy only; laparoscopic treatment of communicating or non-communicating uterine horn. Results: The search strategy identified 45 articles. After this first screening, 37 studies were evaluated. The full text of remaining articles was examined. 35 studies were finally included in this article. All included studies were case reports, due to the rarity of this condition. Rudimentary horns were non-communicating in all cases. Conclusion: The laparoscopic removal of a rudimentary uterine horn could be considered a feasible therapeutic option. An accurate preoperative evaluation is mandatory to assess anatomic variants and to select the optimal and tailored surgical approach, based also on the symptoms complained by the patient.