A 62 -yearold man presented with pain in his lower right abdomen. Computed tomography (CT) revealed a cystic lesion in the ileocecal region. A preoperative blood examination indicated an elevated level of serum CA19 -9 (351.5 U/mL) . The possibility of a malignant tumor in the ileocecal region prompted laparoscopic surgery, which confirmed the presence of a cyst attached to the appendix and a 10 cm ileal duplication located 200 cm from the Treitz ligament. Subsequently, a partial resection of the ileum was performed. Pathological examination of the specimen revealed that the ileal duplication consisted of ileal tissue, exhibiting a fullthickness structure resembling the intestinal tract. Moreover, a connection was established between the cyst and the ileal duplication, confirming their continuity.The patient was diagnosed with a tubular and cystic gastrointestinal duplication without any malignant characteristics. Tubular and cystic gastrointestinal duplications are rare conditions, often accompanied by elevated preoperative tumor markers. The absence of malignancy in such cases is even rarer, prompting this case report alongside a comprehensive review of the existing literature.