Incontinentia pigmenti is a multisystem disorder affecting predominantly females. It is characterized by distinct cutaneous features which classically occur in four stages in the distribution of Blaschko lines: vesicular, verrucous, hyperpigmented and atrophic. These stages often occur in a temporal sequence but all stages do not necessarily occur and several stages may overlap. Additional abnormalities that commonly occur include: dental anomalies such as hypodontia or abnormally shaped teeth, peripheral neovascularization of retina and neurodevelopmental abnormalities. Incontinentia pigmenti is an X-linked dominant disorder caused by mutations in the IKBKG gene (previously known as the NEMO gene). In the majority of affected women, a recurrent deletion in the IKBKG gene is responsible and molecular diagnosis is available. Knowledge of this multisystem disorder and early diagnosis is important for clinical management, particularly because of the risk of neovascularization of retina which can result in visual loss if not detected in time.