The paper presents the coexistence of a nevus flammeus and basal cell carcinoma affecting the left upper lid of 61-year-old farmer with Sturge-Weber syndrome. The occurrence of basal cell carcinoma in nevus flammeus is extremely rare. The etiology of the malignancy in this situation is unknown. It is difficult to diagnose neoplastic transformations that could arise in the nevus flammeus. Radiotherapy followed by complete surgical excision were used as a treatment in the described patient.