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Background: Pilomatrixoma is a benign skin tumor arising from the hair matrix cells. It is typically found in the head and neck region. However, few cases of pilomatrixoma that mimic a pyogenic granuloma have been reported. Clinicians should be aware of it because it may recurrence and transform into a malignant tumor. Case Presentation: We report a rare case of a 9-year-old boy who presented with an asymptomatic mass on the left cheek for 2 months. Clinically, it was diagnosed as a pyogenic granuloma. An excisional biopsy was performed and sent to the pathology department for histopathological study. Histological examination showed that it was a pilomatrixoma. The patient was discharged on the same day, without any complications. Conclusion: Pilomatrixoma is often misdiagnosed clinically; therefore, the definitive diagnosis of skin tumors should be made after a histologic examination of the excisional mass. Surgical resection with wide margins was the optimal treatment for pilomatrixoma because local recurrence may occur.