An 11-year old girl, born at term following an uneventful pregnancy, had some blisters on both sides of the head and discrete but linear vesiculobullous lesions on the arms and the trunk at birth. Months later, she developed hyperpigmented lesions over the areas of the vesiculobullous lesions. She achieved menarche at the age of 7 years and by 9 years, had fully developed secondary sexual characteristics. Her bone age was advanced and had luteinizing hormone and follicular stimulating hormone in the pubertal range. Our patient has demonstrated a rare clinical association of incontinentia pigmenti with idiopathic precocious puberty. The clinical significance of this association of incontinentia pigmenti with precocious puberty is not known. Keywords: Incontinentia pigmenti, precocious puberty Annals of African Medicine Vol. 5(2) 2006: 111-113
