We describe herein a 67-year-old white woman affected with Gorlin-Goltz syndrome. In early adulthood, she developed various odontogenic cysts and multiple basal cell carcinomas (BCCs). Clinical examination revealed multiple small BCCs on the patient's trunk and numerous palmar pits. On her left palm, a waxy, flesh-colored, ulcerated nodule measuring 0.6 × 0.5 cm with telangiectases was observed (Figure 1). The patient reported that this lesion had slowly grown over years.