The immense majority of neuroendocrine neoplasms of the appendix are carcinoid tumors. Most are of enterochromaffin (EC) cell type, although rare examples are of L cell type.New discernmentsoccurred last decade that enriched our knowledge regarding the biological performance of appendiceal neuroendocrine tumors (NETs), which range from totally benign tumors less than 1cm to goblet cell carcinomas which behave correspondingly to colorectal adenocarcinoma. The clinical implication of that knowledge reflected to surgical strategies which also vary from simple appendicectomy to radical abdominal procedures based on specific clinical and histological characteristics. We present a rare case of neuroendocrine tumor of appendix diagnosed after appendectomy in young patient.