Multiple Lentigines (Leopard) Syndrome with Chiara I Malformation

This is a case report of multiple lentigines (Leopard Syndrome) with Chiari malformation, i.e. a herniation of the cerebellum into the foramen magnum. This male patient had generalized multiple lentigines since birth, and the lesions spread steadily, involving the scalp but sparing all mucous membranes. Organ system involvements included heart murmur, ocular hypertelorism, and retardation of growth.