Background <p>Gallbladder (GB) adenocarcinoma accounts for 10% of all cases worldwide and is widespread in the Indian subcontinent, particularly in the Gangetic belt (eastern Uttar Pradesh and Bihar). Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) are mixed epithelial neoplasms characterized by ≥ 30% each of morphologically and immunohistochemically recognizable neuroendocrine and non-neuroendocrine components. GB MiNENs are extremely uncommon cancers with a dismal prognosis, even though they present with comparable symptoms and in the same age range as adenocarcinomas. Since MiNENs are extremely uncommon, specific management and treatment protocols have not been established. Here, we reviewed the relevant literature and presented two cases.</p> Case Presentation <p>Our two patients were postoperatively diagnosed with gallbladder MiNENs. In the literature review, a total of 95 cases of gallbladder MiNENs were identified, including our two cases. GB MiNENs are more common in females (male-to-female ratio of 0.26) with a mean age of 63.5&#xa0;years (<i>n</i> = 95). The mean size of the tumor was 50&#xa0;mm (range: 10 to 150&#xa0;mm,&#xa0;<i>n</i> = 78). Liver involvement was seen in 27 cases (<i>n</i> = 78), while lymph node metastasis was noted in 40 cases (<i>n</i> = 77). Neuroendocrine carcinoma (NEC) without specified sub-classification (<i>n</i> = 30, 31.6%) was the most prevalent neuroendocrine component, followed by small cell neuroendocrine carcinomas (SCNEC,&#xa0;<i>n</i> = 25, 26.3%), large cell neuroendocrine carcinomas (LCNEC,&#xa0;<i>n</i> = 20, 21.1%), and neuroendocrine tumors (NET) (<i>n</i> = 3, 3.2%) including our two cases. Adenocarcinoma was the primary non-neuroendocrine component, but in 3.2% (<i>n</i> = 3) of patients, there was more than one non-neuroendocrine component noted. Intracholecystic papillary neoplasm (ICPN) was noted in one case. When NEC is present, MiNEN frequently behaves more aggressively than the non-neuroendocrine component and exhibits early vascular invasion and distant metastases despite the absence of early symptoms. The survival of MiNEN patients mainly depends on NEC, which is closely related to lymph nodes and liver metastasis.</p> Conclusions <p>Gallbladder MiNENs are extremely rare, with aggressive progression when NEC is present. Radical surgery remains the primary treatment option; however, further research is needed to clarify the roles of biological therapy and debulking in disease management.</p>