Pilomatrixoma, also known as pilomatricoma, is a benign tumor that originates from the matrix of the hair root. It usually presents as a single, slow-growing subcutaneous or intradermal firm nodule with a general size of less than 3 centimeters (cm) in diameter. However, giant pilomatrixomas (more than 5 cm) have been reported infrequently. It is more common in females and usually presents during the first two decades of life (60%) as an asymptomatic, mobile, hard, elastic mass. Most of the cases are benign and affect the face. The authors report a rare case of a giant pilomatricoma of the cheek and discuss the surgical management of these lesions, histopathological findings, and review of the literature.