<p>En coup de sabre scleroderma (ECDS) and progressive hemifacial atrophy (PHA) are classified as forms of linear scleroderma of the head and face, a subset of juvenile localized scleroderma (JLoS). In ECDS, sclerotic lesions primarily localize to the forehead and frontoparietal scalp, typically unilaterally. PHA involves the atrophy of facial soft tissues and adjacent cartilage-bone structures on one side, leading to significant deformities. Both forms may involve the eye and central nervous system (CNS), potentially presenting with hemiplegia in PHA. These conditions can coexist and require immediate, intensive immunosuppressive treatment to halt the disease process. This case report describes the clinical management of a female patient diagnosed with ECDS and PHA at age 5, alongside limited superficial morphea lesions on the face. The patient was treated with high-dose glucocorticosteroids, methotrexate (MTX), cyclosporine A (CsA), and intravenous immunoglobulin (IVIG), achieving transient stabilization. No ophthalmological or neurological symptoms were observed, and brain magnetic resonance imaging (MRI) remained normal. Disease extent was monitored via clinical examination, the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT), and facial MRI assessing skin and subcutaneous tissue thickness. Due to disease progression, tofacitinib was initiated. Over a one-year observation period, stabilization of lesions was achieved with no recorded complications.</p>