We experienced one case of tuberous sclerosis occurred in brothers. Case 1 is 8-year-old male patient who presented seizure, mild mental retardation, subependimal nodule and Shagreen patch. Case 2 is 9-year-old male patient who presented adenoma sebaceum, Shagreen patch, subependymal gient cell astrocytomas. Diagnosis was confirmed by brain CT and biopsy. Brief review and related literatures were also presented.