Intestinal perforation (IP) is a rare complication of systemic lupus erythematosus (SLE), and the timely diagnosis and treatment of IP are necessary to prevent death. In this study, the clinical features of IP in SLE were described in an attempt to enhance its understanding to reduce mortality. The clinical data of IP in SLE from 1984 to 2022 were retrospectively collected. A total of 18 patients were enrolled, and data on clinical symptoms, preoperative evaluation, surgical procedures, and postoperative outcomes were collected and retrospectively analyzed. The analysis included 15 females and 3 males, with a mean age of 49.2 years. Fifteen patients (83.3%) had a history of the disease for >5 years, and the SLE disease activity index score of 1 (5.6%) patient was <5 points and that of 17 (94.4%) patients was >10 points. A total of 9 (50%), 5 (27.7%), 3 (16.7%), and 1 (5.6%) patient had lesions in the rectum, colon, ileum, and both ileum and appendix, respectively. The cause of perforation in 12 (66.7%) patients was lupus mesenteric vasculitis and in 3 (16.7%) patients was chronic inflammation. Seven (38.9%) patients had other immune system diseases. All patients were treated with steroids and surgical treatment. However, 5 patients died after surgery. A disease duration of >5 years, SLE disease activity index score of >10, nonstandard use of steroids, and concomitant presence of other immune system diseases are the possible risk factors of IP in SLE. The most common site of perforation was the rectum, which was caused by lupus mesenteric vasculitis. The results suggest that the key to successfully manage such cases is early diagnosis, aggressive resuscitation, antibiotics, steroid therapy, and prompt surgical intervention.