Background: Pilomatrixoma is a benign tumor of the hair follicle that can transform into a malignant lesion, the pilomatrix carcinoma. We present an unusual case of an eyelid pilomatrixoma. Main observation: A 37-year-old white male presented with rapidly growing, pedunculated lesion located at the superior right eyelid of five months duration. The lesion was excised under local anaesthesia. The sample was fixed in 10% formalin and histopathological as well as immunohistochemical analyses were performed. Results of both examinations were consistent with a benign pilomatrixoma. Conclusions: Even though the lesion had malignant clinical appearance, histopathology confirmed the diagnosis of a benign pilomatrixoma, supporting the decision not to make a more extensive surgery.