ABSTRACT
Incontinentia pigmenti is a genetic disease with cutaneous, dental, neurologic and ophthalmologic manifestations. Ocular changes are very varied. Retinal vasculitis is uncommon, but can be observed in this disease. To report a case of a patient with retinal vasculitis associated to Incontinentia pigmenti. A seven year old girl was followed for cutaneous lesions with the diagnosis of incontinentia pigmenti confirmed by a skin biopsy. She had delayed eruption of her teeth some of which were conical. She had no neurologic nor developmental abnormalities. Ophthalmological examination and fluorescein angiography was performed and showed retinal neovascularization in the left eye, secondary to retinal ischemia and retinal sequelae vasculitis without inflammation or complications in the right eye. Laser photocoagulation was indicated to the left and a strict surveillance to the right eye. After one month of the beginning of treatment, we noticed an involution of the retinal neovascularisation. Early diagnosis and management of retinal changes in incontinentia pigmenti is essential because they may be serious leading to blindness