Background Ladd’s Procedure has been the surgical intervention of choice in the management of congenital intestinal malrotation for the past century. Historically, the procedure included performing an appendectomy to prevent future misdiagnosis of appendicitis, since the location of the appendix will be shifted to the left side of the abdomen. This study consists of two parts. A review of the available literature on appendectomy as part of Ladd’s procedure and then a survey sent to pediatric surgeons about their approach (to remove the appendix or not) while performing a Ladd’s procedure and the clinical reasoning behind their approach. Methods The study consists of 2 parts: (1) a systematic review was performed to extract articles that fulfill the inclusion criteria; (2) a short online survey was designed and sent by email to 168 pediatric surgeons. The questions in the survey were centered on whether a surgeon performs an appendectomy as part of the Ladd’s procedure or not, as well as their reasoning behind either choice. Results The literature search yielded five articles, the data from the available literature are inconsistent with performing appendectomy as part of Ladd's procedure. The challenge of leaving the appendix in place has been briefly described with minimal to no focus on the clinical reasoning. The survey demonstrated that 102 responses were received (60% response rate). Ninety pediatric surgeons stated performing an appendectomy as part of the procedure (88%). Only 12% of pediatric surgeons are not performing appendectomy during Ladd’s procedure. Conclusion It is difficult to implement a modification in a successful procedure like Ladd’s procedure. The majority of pediatric surgeons perform an appendectomy as part of its original description. This study has identified gaps in the literature pertaining to analyze the outcomes of performing Ladd's procedure without an appendectomy which should be explored in future research.