We report an unusual case of a young male with cerebellar hemangioblastoma treated previously for medullary carcinoma of thyroid, whose PET/CT scans revealed a constellation of findings that suggested the rare Von Hippel Lindau syndrome. The diagnosis was clinched by confirming the findings on whole body contrast enhanced computed tomography (CECT) and contrast enhanced magnetic resonance imaging (CEMR). The report highlights the need to carefully evaluate subtle findings on PET/CT that could be missed or misinterpreted as other diagnoses. It also adds to the existing literature of two cases with Von Hippel Lindau syndrome and medullary carcinoma of thyroid.