Von Hippel-Lindau (VHL) disease is a rare, autosomal dominant, multisystem cancer syndrome. A variety of benign and malignant tumors can develop in VHL, including cranial and spinal hemangioblastomas, retinoblastoma, endolymphatic sac tumor, pheochromocytoma, renal cell carcinoma, pancreatic neuroendocrine tumors, and renal, pancreatic, and genital cysts. Here we present a 57-year-old woman who was diagnosed with VHL and, in the same 68 Ga-DOTATATE PET/CT, exhibits all these components of the syndrome: pheochromocytoma, pancreatic neuroendocrine tumor, pancreatic cysts, renal cysts, liver hemangioma, and hemangioblastoma in the medulla oblongata.