Abstract: Stiff skin syndrome (SSS) is a rare hereditary dermatosis. We reported a pedigree of SSS. The proband was a 11-year-old boy and the onset age was three and a half years. The proband presented with stiff skin on the right thigh, and gradually expand to the right calf, right hip, waist and abdomen, with excess vellus hairs on the lesions. At the age of 5, the movement of right knee was limited. The diagnosis of SSS was made according to the clinical manifestation and pathological features. Twenty-seven persons in 4 generations were investigated, and 3 patients (2 males and 1 female) were diagnosed as having SSS. No pathogenic mutation was detected in this family.