Pilomatricoma is a benign tumor arising from hair follicle matrix cells, presenting as an asymptomatic, firm, slow growing, mobile, superficial skin nodule typically in children. This lesion with an atypical clinical presentation is frequently misdiagnosed as other skin lesions and even as malignant entities regardless of detailed cytological, imaging examinations; the site of occurrence is one of the keys to accurate diagnosis. Here, we present a case of pilomatrixoma involving the ear, the cymba conchae of the auricle, which is an extremely rare site for the lesion in a 52-year-old woman. The present case suggests that this benign tumor needs to be included in the differential diagnosis in patients who present with an atypical auricular lesion.