Introduction: Acute appendicitis is one of the most commonly diagnosed surgical diseases in children with emergency abdominal pain. Atypical localized appendicitis is rare and difficult so we can see delay in diagnosis. Malrotation anomaly is also a cause of left-sided appendicitis. Herein, we present a rare case of acute appendicitis in the left lower quadrant with malrotation anomaly.Case Report: A 12-year-old male patient was admitted to the emergency department due to abdominal pain since 2 days. We discovered that the abdominal pain was initially widespread in the abdomen and gradually dispersed to the lower left side. Medical examination and radiological assessment were conducted, and a diagnosis of acute appendicitis in left lower quadrant was made. The patient subsequently underwent surgery and was discharged on the second postoperative day. Conclusion: There are many causes of left lower quadrant pain in the abdomen. In cases with suspected findings on physical examination, imaging methods can be used to diagnose rare incidents