The primary neuroendocrine tumors of the gallbladder are very rare, representing 0.2% of all neuroendocrine tumors. The definite diagnosis is incidental in most cases.A 60-year-old male patient presented with a one-month history of epigastric pain and jaundice, itching, flushing, cough and wheezing. Ultrasound demonstrated the gallbladder containing multiple stones, with wall thickening and an adjacent liver lesion. Additionally, those structures showed noticeable contrast enhancement in CT and MRI. Histopathological examination revealed a primary gallbladder neuroendocrine tumor.Though a rare entity, primary neuroendocrine tumor should be considered in the differential diagnosis of gallbladder cancers.