Basal cell carcinoma (BCC) is the most common type of skin cancer in the Caucasian population. However, BCC of the genitalia is extremely rare. To the best of our knowledge, only 30 patients with BCC of the genitalia have been reported in the literature so far. BCC usually occurs in individuals over the age of 50, on sun-exposed areas of the body, and it is more prevalent in females. Superficial BCCs account for approximately 10% of BCC, and are commonly found on the trunk or limbs. This is a report on a 55-year-old male patient with penile BCC, and a literature review on this condition. The patient presented with asymptomatic skin lesions in the genital area which progressed during the last 15 years. Before admission, the patient received: antimycotics, antiviral, antibacterial and corticosteroid therapy, but without any improvement. Examination of the proximal penile skin revealed a sharply marked, smooth and shiny erythematous plaque of about 3.3 x 2 cm, with whitish squamae in the central zone. The histopathology finding revealed a superficial BCC and total excision was recommended. Treatment of the superficial BCC depends on its size and location. It includes surgical removal with possible reconstruction, radiotherapy, application of immunomodulators, local chemotherapy and local retinoid therapy. Although this is an extremely rare type of BCC, it is important to keep in mind that it may affect body areas seldom exposed to direct sunlight.