Background and Objectives: Cornual pregnancies (CPs) are rare forms of ectopic pregnancy. When abortion does not occur, it can be a life-threatening condition for the mother and can also impair future fertility. We present our experience in the diagnosis and management of CPs. A systematic review was also conducted to investigate the reproductive outcomes after treatment. Materials and Methods: Between January 2010 and December 2022, we performed a retrospective, cross-sectional, single-center, and descriptive data collection and analysis (ClinicalTrial ID: NCT06165770). The search for suitable articles published in English was carried out using the following databases (PROSPERO ID: CRD42023484909): MEDLINE, EMBASE, Global Health, The Cochrane Library (Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, and Cochrane Methodology Register), Health Technology Assessment Database, Web of Science, and search register such as ClinicalTrial. Only studies describing the impact of CP treatment on fertility were selected. Results: Two studies were included in the systematic review. Seventeen patients suffering from CPs were selected. In our series, a pelvic ultrasound allowed for the diagnosis of a cornual localization in 35.30% of cases. Thirteen women (76.47%) underwent immediate surgical management. The laparoscopic approach was the most used (76.92%), with a laparotomic conversion rate of 30%. Four patients (23.52%) received medical treatment with methotrexate. After treatment, two patients managed to achieve pregnancy. Conclusions: CP is a rare form of ectopic pregnancy that can quickly become life-threatening for the mother. Ultrasound does not lead to a precise diagnosis in all cases. In the absence of complications and emergencies, laparoscopy is an approach that could be considered valid. For selected asymptomatic patients, medical treatment may be a valid alternative. The data from the studies included in the systematic review, although demonstrating a superiority of medical treatment in terms of future pregnancies, are heterogeneous and do not allow us to reach a definitive conclusion.