Nevus sebaceus is a common hamartoma that has a variety of appendage tumors. We report a case of nevus sebaceus with multiple appendage tumors on the scalp of a 43-year-old male. The exudative inflamed patches were admixed with darkly pigmented small nodules which had developed secondarily within a 4.0 × 1.5cm lesion 2 years previously. Histologically, on serial sectioning, there were various types of basal cell carcinoma, syringocystadenoma papilliferum, tubular apocrine adenoma and sebaceous adenoma. Some parts of a biopsy specimen, showed a tumor of the follicular infundibulum-like epidermal changes. There were also calcium depositions in the stroma and apocrine tumors, There was no relapse at 1-year follow-up after surgical treatment.