The coexistence of adenocarcinoma and neuroendocrine neoplasm (NEN) in the same tumor is rare. What is rarer is that the neuroendocrine component is a well-differentiated neuroendocrine tumor (NET) Grade (G) 1. Most colorectal NETs are single, but multiple neuroendocrine tumors (M-NETs) are rare. Well-differentiated NETs rarely metastasize. Here, we present a unique case of a synchronous sigmoid tumor and multiple colorectal NETs with lymph node metastases. The sigmoid tumor consisted of adenocarcinoma and NET G1. The metastatic component was NET G1. A 64-year-old man underwent a colonoscopy for persistent changes in bowel habits and positive fecal occult blood for 1 year. An ulcerative lesion, which was diagnosed as colon cancer, was seen in the sigmoid colon. In addition, scattered lesions could be seen in the colon and rectum. Surgical resection was performed. Pathological findings suggested that the ulcerative lesion was composed of 80% adenocarcinoma and 20% neuroendocrine component (NET G1), while the remaining lesions were consistent with NET G1. At the same time, 11 lymph nodes around the resected intestinal segment were invaded by NET G1. The prognosis of the patient was good. After 13 months of follow-up, no recurrence and no metastasis were found. We hope to provide a reference and improve our understanding of the clinicopathological features and biological behavior of these unique tumors. We also aim to emphasize the importance of radical surgery and individualized treatment.