Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytic disorder. The most common manifestations consist of polyostotic sclerotic lesions with the majority of cases also demonstrating soft tissue involvement of the sinuses, retroperitoneum, large vessels, heart, lungs, and central nervous system. Nuclear medicine can play an important role in assessing the extent of the disease with bone scintigraphy and fluorodeoxyglucose (FDG)-positron emission tomography (PET). We present the case of a middle-aged female who initially presented with tooth pain. She subsequently underwent imaging including plain film, bone scan, computed tomography (CT), magnetic resonance imaging (MRI), and FDG-PET/CT, which showed characteristic bony involvement. Biopsy results confirmed the diagnosis of ECD.