There will be a brief case presentation highlighting the clinical features of a malignant insulinoma representing a very rare functional pancreatic neuroendocrine tumor (pNET). The relevant diagnostic biochemical, imaging, and immunohistochemical features will be presented. The video will point out the specific epidemiological characteristics of malignant insulinomas including treatment options and prognostic factors. Particular emphasis will be focusing on the role of somatostatin receptors in theranostics of pNETs and the mutational profiling of NETs originating from various anatomical locations. The novel molecular findings in this case will be stated and put into context of the current molecular landscape of pNETs with potential novel treatment targets.