Pilomatricoma is one of the rare skin benign neoplasms arising from the matrix cells of hair follicles. We report the case of a 17-year-old female who had a swelling over the medial surface of the right ear lobule, which was determined to be an epidermal inclusion cyst by radiological and clinical examination. Over the period of eight days, it grew to 2 × 2 cm. A well-defined encapsulated lesion with thick cellular debris was found on an ultrasonography of the local region. Under local anesthesia, the patient had a cyst excision. Early detection by ultrasonography is beneficial in skin lesions to confirm if they are benign or malignant. Complete excision of the cyst is the treatment in order to limit the morbidity and lower the aggressive behavior. In this case, we came to a diagnosis after a histopathological examination, confirming it as a pilomatricoma. Due to its rarity, it is often misdiagnosed.