Research in the field of genetic skin disease has grown rapidly over the past two decades. Even though the fundamental molecular pathways are still not fully understood, there have prominent advances in our understanding of the underlying mechanisms involved in the pathogenesis of genodermatosis. Dermatologists in China contributed to this field in recent years. They found the causative genes involved in primary erythermalgia and familial trichoepithelioma. Different gene mutations involved in more than twenty kinds of genodermatosises have been detected. This review will synthesize recent findings of the genodermatosises in China.