A pilomatrixoma is a benign tumor originating from a hair follicle that most frequently occurs in the head-and-neck region. It usually presents as a subcutaneous, slow-growing, nodular, painless firm mass. There are few reported cases on eyelid pilomatrixoma. We reporting an unusual presentation of a rapidly growing pedunculated eyelid pilomatrixoma in a 29-year-old female patient. Surgical excision was performed, and histological examination showed a cavity containing proliferating cords of basaloid cells differentiated into eosinophilic keratinized shadow cells confirming the pilomatrixoma diagnosis. Only a few cases with pedunculated eyelid masses have been reported in the literature; pedunculated lesions can be misdiagnosed as vascular tumors or malignancies. Therefore, pilomatrixoma should be considered in the differential diagnosis of such a presentation. A complete excisional biopsy of the mass is diagnostic and therapeutic.