Linear scleroderma is the most common type of juvenile localized scleroderma, which usually involves the limbs. The linear lesions may extend through the dermis, subcutaneous tissue, muscles, and the underlying bone, leading to significant functional and cosmetic deformities. Renal involvement in juvenile localized scleroderma is a rare manifestation. Here we report two cases with juvenile linear scleroderma and unique renal manifestations, including unilateral renal arterial stenosis and immunoglobulin M nephropathy.