We present the case of 60-year-old man (twin) with ileus cause by intussusception of the caecum adenocarcinoma and coexistence of intestinal malrotation (IM) and agenesis of one kidney. Intestinal malrotation with colon carcinoma is very rare in adult patients (only 14 cases have been described in the literature). Ileus cause by tumour intussusception has been previously reported in only one patient and the additional coexistence of agenesis of one kidney has not been reported in the available medical literature. However, coexistence of other anomalies was observed in 46 per cent of patients with IM. Therefore, in patients with ileus and coexisting congenital anomalies in other organs, the existence of intestinal malrotation should be considered. Furthermore, because of the unusual anatomy of the mesenteric vessels, right colon resection in patients with IM should be performed with special caution and the preoperative work-up should be extended to include visceral angiography.