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
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F-fluorodeoxyglucose positron emission tomography-computed tomography (
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F-FDG PET/CT) scan was requested for staging, revealing marked FDG uptake restricted to the primary lesion and no evidence of separate disease. The purpose of this report is to demonstrate the importance of PET/CT in the staging of this FDG-avid tumor; the malignancy of which is often first revealed by metastases. Our case also demonstrates that pilomatricoma should be included in the differential diagnosis of a rapidly growing peripheral soft-tissue mass; conventionally, the domain of sarcoma.