Here we described a rare case of insulinomatosis with novel CNVs that are seen in multiple micro-tumors and a macro-tumor and harbor CEBPA, FOXL2 and IRS2 genes that can be involved in pancreatic neuroendocrine tumor pathogenesis, specifically insulinomatosis, and can provide new insights into the disturbance of morphogenesis of β-cells. This evidence concerns the gene IRS2 and pancreatic neuroendocrine tumor.