Speckled lentiginous nevus syndrome is a recently described condition, characterized by unilateral neurocutaneous manifestations consisting of: speckled lentiginous nevus, hyperhidrosis, muscular weakness and dysesthesia. Skeletal anomalies such as scoliosis, limb asymmetry, and psychiatric manifestations have been also reported. Herein, we report a case of speckled lentiginous nevus syndrome with skeletal anomalies and a pseudo-marfanoid phenotype.