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The presence of a functional syndrome must be sought when diagnosing a pancreatic neuroendocrine tumor, because its complications can be life-threatening, impair quality of life, and impact survival. When a functional syndrome is suspected, hormonal and/or peptide assays must be performed urgently, concomitantly with the topographical diagnosis of the neuroendocrine tumor and the staging assessment. Specific antisecretory treatment is often urgent: diazoxide for insulinoma, high-dose proton pump inhibitors for gastrinoma, somatostatin analogues for VIPoma or glucagonoma. Curative resection should always be considered in cases of localized and sporadic functional pancreatic neuroendocrine tumor. Management is multimodal, combining dietary measures and nutritional support, patient education, the antisecretory treatment, and an antitumor treatment. This mini review provides an update on the diagnosis and management of the main functional pancreatic neuroendocrine tumors.