Nevus sebaceous (NS) is a benign congenital hamartoma with the potential to develop into a secondary benign or malignant skin tumors. Two or more skin tumors rarely arise simultaneously from a single NS lesion. Herein, we report a case of syringocystadenoma papilliferum and basal cell carcinoma arising in an NS, based on dermoscopic, reflectance confocal microscopic, and histopathological findings.