Background: Umbilical hernia is a common and potential serious condition in patients with cirrhosis. This systematic review evaluated the risks associated with emergency and elective hernia repair in patients with cirrhosis. Methods: Systematic review of clinical trials identified through manual and electronic searches in several databases (last update November 2021). The primary random-effects meta-analyses evaluated mortality in patients with or without cirrhosis or following emergency versus elective repair. The quality of the evidence was assessed using GRADE and Newcastle Ottawa Scale. Results: Thirteen prospective and 10 retrospective studies including a total of 3229 patients were included. The evidence was graded as very low quality for all outcomes (mortality and postoperative complications within 90 days). In total 191 patients (6%) died after undergoing umbilical hernia repair. Patients with cirrhosis were more than eight times as likely to die after surgery compared with patients without cirrhosis [OR = 8.50, 95% CI (1.91–37.86)] corresponding to 69 more deaths/1000 patients. Among patients with cirrhosis, mortality was higher after emergency versus elective repair [OR = 2.67, 95% CI (1.87–3.97)] corresponding to 52 more deaths/1000 patients. Postoperative complications were more common in patients with cirrhosis compared with patients without cirrhosis. Conclusion: Patients with cirrhosis undergoing emergency umbilical hernia repair have a considerably increased risk of death and severe complications. Accordingly, additional evidence is needed to evaluate methods that would allow elective umbilical hernia repair in patients with cirrhosis.