This chapter provides detail description of Autoimmune connective tissue diseases in children. Chapter has 8 well illustrated diagrams and pictures. Autoimmune connective tissue diseases in children, juvenile systemic sclerosis, localized scleroderma, morphae, linear scleroderma, juvenile dermatomyositis, amyopathic dermatomyositis, systemic lupus erythematosus (SLE), neonatal lupus erythematosus (NLE), cutaneous manifestations of chronic arthritis in children are described in detail. 3 tables are included to highlight clinical features, classification of SLE and classification of lupus nephritis. 4 boxes are given to provide key points of juvenile systemic sclerosis, linear scleroderma, juvenile dermatomyositis and SLE. 113 references are included at the end of the chapter to facilitate further reading.