Hepatodiaphragmatic interposition of the colon, known as Chilaiditi's sign, is generally discovered by chance, during an x-ray study for a different cause as its appearance usually lacks symptoms. When the discovery is accompanied by clinical symptoms such as: abdominal pain, nausea, vomiting, constipation, it is known as the Chilaiditi's syndrome. Transverse colon volvulus is a rare entity. The treatment is emergency surgery. The association of Chilaiditi syndrome and transverse colon volvulus is exceptional, the presence of elongation and hypermotility of the colon associated to long mesenterium are common findings in patients with this association. The presence of signs and symptoms compatible with intestinal obstruction in this clinical association, change the conservative medical handling described classically in the Chilaiditi syndrome. Based on the above, the conduct was surgery for the benefit of the patient. We presented the seventh case in the English world medical literature and the first in Peruvian medical literature, in a 17 year old mentally retarded male patient with renal ectopia.