We report a case of a congenital nevus sebaceus with numerous brown macules and papules in the left auricular region. In the same lesion, pigmented basal cell carcinomas developed later in life. Additionally, on the left side of the neck, both shoulders and the left arm, features of nevus spilus with melanocytic and blue nevi were present. Such a combination of nevus sebaceus with melanocytic lesions may be explained by the concept of twin spotting and represent another case of phacomatosis pigmentokeratotica.
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