Abstract: IntroductionDowling-Degos disease (DDD) was first described as a benign form of acanthosis nigricans by Dowling and Freudenthal in 1938, then referred to as"dermatose reticulée des plis"by Degos and Ossipowski in 1954, and was first named DDD by Wilson-Jones and Grice in 1978[1]. The disease has been reported worldwide and affects both genders equally. As an autosomal dominant pigment disorder, it usually occurs in post-pubertal individuals, and is seldom seen in children[2]. However, a Chinese newborn with DDD was reported in 2008[3]. In this review, we summarize features of DDD, emphasizing advances in genetics research and looking to the future for further understanding of its etiology and the development of therapeutic methods.