Extra-appendiceal neuroendocrine expressing goblet cell adenocarcinoma represents a challenging diagnosis; through our case report, we aimed to raise awareness about possible extra-appendiceal localisations of GCAs by presenting a high-grade goblet-cell adenocarcinoma of the cecum, with positive stains for synaptophysin and neuronal specific enolase and MUC 2 immunoreactive goblet cells. Here, ENO2 is linked to goblet cell carcinoma.