: Gastroenteropancreatic neuroendocrine tumors (GEP NET) represent a heterogenous group of neoplasms: carcinoids (serotoninomas) and gastroenteropancreatic (insulinomas, gastrinomas, VIPomas, glucagonomas, somatostatinomas) respectively, unified by their origin (neuroendocrine cells), histology and immunohistochemi- cal profile. Unlike their frequency in adults, the rarity of these lesions in childhood makes difficult their early diag- nosis. Many tumors can be asymptomatic or may show non-specific features, the diagnosis being nevertheless based on clinical signs, dosage of hormonal specific pep- tides, nuclear medicine imaging and pathology confirma- tion. Baseline tests should also include chromogranine A and sinaptophysine. Localising studies comprise CT, MRI, somatostatine receptor scintigraphy and ultrasonography completed by endoscopy. Surgery is the mainstay therapy of GEP NET, as a complete removal can potentially cure the disease; debulking and metastasis surgery, together with adjuvant medical therapy can alleviate some symp- toms, sometimes for a long period. Survival is variable, depending on tumour’s type, stage, histology and also on the completeness of the treatment.