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Abstract: Metastatic pituitary neuroendocrine tumors (PitNETs) are rare and often aggressive. Patients should be evaluated for metastatic disease when their biochemical presentation is discordant with pituitary tumor burden or when they present with non-pituitary, site-specific symptoms. Here, we describe the case of a 55-year-old male with an ACTH-secreting PitNET and more than a decade of recurrent transsphenoidal resections, gamma knife irradiation, and therapy with somatostatin analogs and steroidogenesis inhibitors before uncovering metastatic osseous deposits. Only after initiation of peptide receptor radionuclide therapy (PRRT) with Learning points: Patients with aggressive, recurrent PitNET and discordant biochemical and structural data or site-specific symptoms should be evaluated for evolution of disease, including metastases.There are multiple alternative agents beyond cytotoxic chemotherapy for progressive metastatic PitNETs, including non-endocrine targeted therapies, immunotherapy, and peptide receptor radionuclide therapy (PRRT).PRRT has successfully been used in patients with metastatic PitNET without significant adverse events while stabilizing tumor burden and clinical hormone excess.