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Sigmoid volvulus (SV) is an exceptionally rare but potentially life-threatening condition in children.Abdominal distention for 1 week.Sigmoid volvulus.We present a case of a 12-year-old boy with mechanical ileus who was finally confirmed to have SV with the combination of abdominal plain film, sonography, and computed tomography (CT) with the finding of mesenteric artery rotation.Because bowel obstruction was suspected, abdominal plain film, sonography, and CT were performed. The abdominal CT demonstrated whirlpool sign with torsion of the sigmoid vessels. In addition, lower gastrointestinal filling study showed that the contrast medium could only reach the upper descending colon. Therefore, he received laparotomy with mesosigmoidoplasty for detorsion of the sigmoid.The postoperative recovery was smooth under empirical antibiotic treatment with cefazolin. A follow-up lower gastrointestinal series on the seventh day of admission showed no obstruction compared with the previous series. He was finally discharged in a stable condition 8 days after admission.SV is a congenital anomaly and an uncommon diagnosis in children. Nevertheless, case series and case reports of SV are becoming more prevalent in the literature. Failure to recognize SV may result in life-threatening complications such as sigmoid gangrene/perforation, peritonitis, sepsis, and death. Thus, if the children have persistent and recurrent abdominal distention, abdominal pain, and vomiting, physicians should consider SV as a “do not miss diagnosis” in the differential diagnosis.