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A brief review of literature and description of an extremely rare case of duodenum glomic tumour are presented. The tumour of the duodenum was detected roentgenologically and endoscopically in a woman of 51, 3 months after the appearance of weakness, pains, and black stool. Pancreaticoduodenal resection was performed. The tumour, 17 X 15 X X 6 cm in size, coming from the duodenum wall and growing into the choledochus wall, was found. Histologically, the tumour consisted of bundles and knots of elongated cells and roundish cells with clear perinuclear zones. Invasive growth into the choledochus wall, polymorphism, mitoses, necroses, vascularity were noted. The death resulted from bleeding 23 days after the operation. No cases of malignant glomic tumours of the duodenum could be found in the literature.