Piebaldism is a rare genodermatosis caused by KIT mutations. We report the case 
of a 5-year-old boy who had the white forelock and leukoderma of piebaldism, but 
the presence of many café-au-lait macules and axillary and inguinal freckling 
complicated the diagnosis. Patients with similar cutaneous findings have been 
previously reported, and their disorder has been attributed to an overlap of 
piebaldism and neurofibromatosis type 1. Legius syndrome is a recently described 
syndrome caused by Sprouty-related, Ena/vasodilator-stimulated phosphoprotein 
homology-1 domain containing protein 1 (SPRED1) mutations that also has multiple 
café-au-lait macules and intertriginous freckling. Based on our current 
understanding of KIT and SPRED1 protein interactions, we propose that 
café-au-lait macules and freckling may be seen in some patients with piebaldism 
and does not necessarily represent coexistence of neurofibromatosis type 1.