Basal cell carcinomas (BCC) are tumors arising from pluripotent stem cells of the epidermis or hair follicles. There are many histological subtypes such as clear cell, a rare variant, and they have the capacity to differentiate towards distinct directions such as neuroendocrine differentiation (NED). An 81-year-old female patient presented with a mass on the lateral side of the right eyebrow. Excisional biopsy was performed. Histopathologic examination revealed clear cell BCC with prominent rosette-like structures suggesting NED. Immunohistochemically, tumor cells were positive for chromogranin and CD56. This is the first reported case of clear cell basal carcinoma with NED.