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Pilomatricoma, also known as calcifying epithelioma of Malherbe, is a rare skin tumor originating from the hair follicle matrix. We report a case of pilomatricoma in a 50-year-old woman, presenting as a rapidly growing pretibial mass. Malignant pilomatricoma is associated with potentially fatal metastases and are clinically and histologically indistinguishable from benign pilomatricoma. Thus, an 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET/CT) scan was requested for staging, revealing marked FDG