Pilomatrixoma, or Malherbe’s calcifying epithelioma, is a benign neoplasm formed from hair matrix cells. It mainly occurs in the head, neck and upper torso area, as a single hard, subcutaneous, non-painful lesion, usually reaching 1 to 5 cm in diameter. Most commonly pilomatrixoma is covered with unchanged skin, rarely with reddish-blue skin discolouration and ulceration. Even though this is a benign tumor, there are cases of transformation to malignant pilomatrix carcinoma. Clinically, it is difficult to distinguish between the lesions so a definitive diagnosis is made on the basis of the result of the histopathological examination. The differential diagnosis should consider a sebaceous cyst, epidermoid cyst, epithelioma, neurofibroma, reaction to foreign bodies, calcified cysts or haemangiomas, cartilage, fibrosarcoma, osteoma cutis and giant cell tumour among others. The authors present the case of a 31-year-old female patient with a nodular lesion in the left auricular region, which lesion was originally treated by a dermatologist.