ABSTRACTAlthough basal cell carcinoma (BCC) is a common skin tumor, very rare cases of BCC arising from upper vermilion mucosa of lip have been reported previously. This tumor basically, originates from pillar structures and the involvement of the vermilion lip contrasts this concept so it is devoid of hair follicles and sweat glands. The exact pathogenesis of vermilion lip BCC is not clear but it has been postulated that the neoplasm originates from the pluripotential epithelial cells of the oral mucosa and epidermis. On the other hand, some authors consider their origin from ectopic sebaceous glands. Herein, we report a 34- year-old man with an asymptomatic ulcerated lesion on the upper left lip vermilion mucosa .The diagnosis of BCC was confirmed with histopathological examination after incisional biopsy of the mucosal neoplasm. After surgery of lip lesion, no recurrency was seen after 3 months follow-up the patient.