Pilomatrixoma is a benign tumor originating from the matrix of hair follicles. It is commonly found in children and young adolescents as a slowly-growing nodule, mostly in the head and neck regions. It is relatively less common in the upper extremities. Pilomatrixomas are usually asymptomatic, solitary, hard, and mobile dermal and subcutaneous nodules and are mostly misdiagnosed despite being commonly seen in the practise. Most are smaller than 3 cm and usually around 1 cm in size while those larger than 5 cm are called pilomatrixoma and are less common. We present a seven-year-old girl with a giant pilomatrixoma on the upper arm in this study.