BACKGROUND Tuberous sclerosis complex (TSC; Bourneville-Pringle disease) is a multisystem genetic disorder manifesting as benign tumors that can affect any system. Malignant neoplasm may coexist in patients with TSC. In such cases, there are diagnostic difficulties in distinguishing between metastatic lesions and benign changes. We show the usefulness of positron emission tomography (PET) in resolving these difficulties. CASE REPORT The purpose of this article is to present the usefulness of metabolic imaging using 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) in distinguishing benign from neoplastic lesions in a patient with TSC. A 17-year-old female patient with TSC was referred for 18F-FDG PET/CT with suspected lung and bone metastases. The patient underwent a bilateral nephrectomy because of multiple cysts and angiomyolipomas. A colonoscopy - performed in preparation for kidney transplantation - revealed sevearal colon polyps, one of which was found to be cancerous upon histopathologic examination. A diagnosis of adenocarcinoma G3 was made and a CT scan of the chest and abdomen performed afterwards showed multiple pulmonary nodules and sclerotic bone lesions suggestive of metastases. Two 18F-FDG PET/CT scans (performed within 6 months) showed multiple nodules of 7-15 mm in diameter and changes typical of multifocal micronodular pneumocyte hyperplasia in both lungs. In the bones, we found multiple sclerotic lesions. All of the above findings showed FDG uptake at the level of the background activity which contradicted the lesions' metastatic origin. CONCLUSIONS Using the example of a 17-year-old patient with TSC, we present the usefulness of metabolic imaging using 18F-FDG PET/CT in distinguishing benign from neoplastic lesions.