Appendicitis is a common surgical abdominal disease with various presentations. Its diagnosis may be obscured by asymptomatic congenital anatomical anomalies like midgut malrotation. Midgut malrotation is a rare fetal anomaly resulting from incomplete or failure of midgut rotation and fixation. It is mostly presented with bowel obstruction or volvulus in early life. Presentation in adult is rare. Here, we report an elderly patient presented with left lower abdominal pain and urinary tract infection. Abdominal computed tomography revealed left-sided appendicitis with non-rotational-type midgut malrotation. Clinicians should bear in mind the possibility of underlying midgut malrotation, as appendicitis could be the first presentation of this rare congenital condition.Keywords Acute appendicitis . Left-sided appendicitis . Midgut malrotation . Intestinal malrotation A 77-year-old female with diabetes presented to our emergency department with poor appetite, lower abdominal pain, and dysuria for 3 days. Physical examination revealed whole abdominal tenderness with mild rebounding pain, especially in the left lower abdomen. Laboratory examination showed no leukocytosis, but hyperglycemia, elevated C-reactive protein (32 mg/dl), and pyuria (urine WBC>100/HPF). An abdominal plain film only showed some large bowel gas in the left hemiabdomen. Because of the progression of abdominal pain, a computed tomography (CT) was done and incidentally found a congenital midgut malrotation with characteristics of transposition of the superior mesenteric artery (Fig. 1a-c, red arrows) and vein (Fig. 1a-c, white arrows), left-sided lying colon, and right-sided lying small intestine (Fig. 1c). A swollen appendix with fecalith and periappendiceal fat stranding were also found (Fig. 2a, b, red arrows). Emergency operation revealed ruptured acute appendicitis with peritonitis. The histopathologic finding of the resected appendix revealed a suppurative stage of acute appendicitis (Fig. 3). After appendectomy with drainage and antibiotics treatment, the patient was discharged uneventfully 8 days later.Midgut malrotation is a rare fetal anomaly resulting from incomplete or failure of midgut rotation and fixation during embryonic development [1]. Most cases have been estimated to present in their early life, and less than 0.5 % of cases are identified in adulthood [2]. It has been classified into three major types, including non-rotation (type I), duodenal malrotation (type II), and combined duodenal and cecal malrotation (type III), according to the stage at which the embryologic error occurs [3,4]. This present patient is in the nonrotation type (type I), which is characterized by right-