Incontinentia pigmenti (IP) is a rare multisystemic X-linked dominant genetic disorder characterized by specific skin lesions. The disease can be misdiagnosed in infants and may have severe complications affecting the eyes and/or the brain. European Network for Rare Skin Diseases (ERN-Skin) issued multidisciplinary consensus recommendations for the diagnosis and practical management of patients with incontinentia pigmenti in March 2020, which put forward the guidance of the diagnosis, treatment, follow-up, etc. for incontinentia pigmenti. This paper interpreted the consensus to provide references for the diagnosis and management of incontinentia pigmenti.