Albright syndrome should be suspected when large, unilateral café-au-lait spots with irregular borders occur in a segmental/Blaschkoid distribution Characteristics of this syndrome are bony abnormalities and precocious puberty Children who have large, swirling areas of pigmentation following lines of Blaschko (lines representing planes of cutaneous embryogenesis) may have forms of cutaneous mosaicism as can be seen in linear and whorled nevoid hypermelanosis Melanocytic lesions are also important in the neonatal period True malignant melanoma is rare in children but can occur Melanoma can arise de novo or within a congenital melanocytic nevus Giant congenital melanocytic nevi have a high risk of malignant transformation, whereas small and medium have a much lower risk of transformation Patients with giant congenital melanocytic nevi are also at an increased risk of neurocutaneous melanosis ( Fig 69.18 ) FIGURE 69.18 Congenital melanocytic nevus Giant congenital melanocytic nevus on the thigh of a neonate