Abstract: Gastric neuroendocrine carcinoma (G-NEC) is a rare malignancy, constituting a small percentage of all gastric cancers. G-NEC is typically diagnosed at advanced stages and exhibits more significant invasiveness than gastric adenocarcinoma, with a higher propensity for vascular invasion, nerve infiltration, and postoperative liver metastasis. Current treatment options are limited and lack standardization, leading to a poor prognosis. Recent studies have elucidated the genomic landscape of human G-NEC, identifying MYC as a pivotal oncogenic driver and establishing a murine model to investigate its molecular and cellular characteristics. Genetic and pharmacological screening has yielded novel insights into the biology and therapeutic approaches for G-NEC. Currently, there is a lack of comprehensive knowledge regarding the pathogenesis, molecular biological characteristics, clinical pathology, and diagnosis and treatment of G-NEC. To advance the systematic comprehension of this condition, this article aims to systematically review domestic and international literature, with the goal of offering theoretical support for large-scale clinical translational research on G-NEC.