Objective: To evaluate the clinicopathological features and literature review of Nevus Sebaceous. Methods: 5 cases of Nevus Sebaceous were investigated by hematoxylin-eosin, the clinical history and pathlogical features were analyzed. Results: In 3 cases (60%) nevus sebaceous occurred during birth, male and female proportion was 3:2. The main pathological features were decreased or disappeared of hair follicles, mature or near mature sebaceous gland hyperplasia were seen, parts of sebaceous gland were directly communicated with follicular infundibulum or epidermis, focal apocrine gland were present below the sebaceous glands. Conclusion: Nevus Sebaceous often presents at birth and commonly affects the head and neck area. Nevus sebaceous is complex lesion comprising abnormalities of the epidermis, hair follicles, sebaceous and sweat glands, and frequently complicated by development of various other cutaneous neoplanms.