Neuroendocrine tumors and rare gastrointestinal malignancies represent a diverse and complex group of diseases with variable clinical behavior. Improved awareness, advanced imaging, and biomarker development have led to increased detection. Accurate classification and staging are essential for appropriate management. Treatment often requires individualized strategies involving surgery, endoscopic therapy, systemic treatment, and targeted approaches. Long-term surveillance is critical due to the risk of recurrence and progression. This topic addresses diagnostic challenges, evolving therapeutic options, and multidisciplinary management of rare GI cancers.