Abstract: A case of linear and whorled nevoid hypermelanosis is reported.A 20-year-old male patient developed hyperpigmented macules over the trunk and extremities soon after birth,which progressively increased.The lesions were arranged in streaks or whorles.Mucous membranes,eyes,palms and soles were spared.Dermatological examination revealed generalized,light tan or deep brown,broad-stripe hyperpigmentation along the Blaschko's lines,which were arranged in streaks on the extremities and in whorles or are shape on the trunk.Skin biopsy revealed increased pigmentation of the basal layer without incontinence of pigmentation.He was diagnosed with linear and whorled nevoid hypermelanosis,and received one-session treatment with Q-switched laser (532 nm),but no obvious improvement was observed.The patient was still followed up.This disease should be diferenfiated from other pigmented disorders,such as incontinentia pigmenti and early linear epidermal nevi.