Incontinentia pigmenti (Bloch-Sulzberger Syndrome) is a rare genodermatosis. The disease is X-linked and the most common molecular defect includes mutations in the NEMO gene on chromosome Xq28 in approximately 70% of patients. It is characterized by a multisystem compromise in which the skin manifestations occur in all patients. Also, the dermatological findings occur early and in chronological order. The prevalence of extra skin compromise is approximately 80% of patients. The ophthalmological and neurological manifestations are the major cause of disability in patients and may have a great impact on the quality of life. Some of damages are irreversible. We present the clinical case of a child on her first day of life. It began with skin manifestations typical of incontinentia pigmenti and extensive neurological involvement. In addition, the family history includes a sister with the disease. A multidisciplinary approach is necessary to care for patients and to detect and prevent long-term complications.