Diagnosis of ectopic Cushing syndrome is challenging. The best imaging approach for localizing ectopic ACTH-secreting tumors is not defined. Here, we report on a 68-year-old woman with new-onset hypertension (>200/90 mm Hg) who was referred to our institution with suspicion of ectopic ACTH-secreting tumor for further work-up. Combined FDG PET/CT as a whole-body imaging modality revealed a neuroendocrine tumor of the pancreatic tail confirmed by surgical exploration.