Pilomatricoma is a benign skin tumor with a variety of presentations, making it difficult to recognize. Its rarity further complicates diagnosis, as it is often mistaken for a lipoma or an epidermal inclusion cyst. Although its etiology remains unclear, early identification is crucial due to the risk of malignant transformation into pilomatrix carcinoma. Here, we report the case of a 41-year-old male with a large pilomatricoma on the upper right arm, emphasizing the possibility of this rare tumor arising in an uncommon demographic.