Adenoid cystic carcinoma (ACC) is a rare neoplasm and the most frequent pathology occurring in the minor salivary glands. Lung, bone, liver, and brain are the most frequent metastasis sites in ACC cases. To our knowledge, cecal and ileum metastasis from ACC has not been previously reported in literature. Herein, we reported a case of cecal and small bowel metastasis (ileum) in a patient with ACC. A 41-year-old male patient with ACC developed iron and Vitamin B12 deficiency anemia during further controls. Lower gastrointestinal (GI) endoscopy detected an ulcerated polypoid lesion with raised margins in cecum. Pathological evaluation of polyp biopsy was consistent with metastasis from ACC. The patient underwent surgery of right hemicolectomy. Pathological examinations revealed an ACC of cecum and small bowel (ileum) showing positive staining with CD117, CK7, and panCK. We aimed to emphasize that performing GI stromal screening in ACC patients presenting with iron deficiency is important for early diagnosis of metastasis.