ABSTRACT Juvenile scleroderma (JS) is a rare chronic connective tissue disorder characterized by progressive fibrosis of the skin and soft tissues with/without internal organ involvements. Scleroderma manifests itself in both systemic (SSc) and localized (LS) forms. This case report aimed to present an Iranian girl with coexistence of SSc and LS treated by Tocilizumab. A 12‐year‐old girl was referred to the rheumatology clinic with the chief complaint of asymmetrical facial appearance starting from two years ago and coexisting with restricted phalangeal motion for the last 6 months. In the physical examination, she had left hemi‐facial atrophy as well as stiffness, swelling, redness, shiny appearance of the skin of hands and feet, and phalangeal joints restriction. Diagnostic assessments, including relevant consultations and laboratory tests, showed nothing important except for slightly elevated erythrocyte sedimentation rate (ESR), C reactive protein (CRP), and antinuclear antibody (ANA) titer of 1/80. At first, the therapeutic intervention consisted of methotrexate, Cellcept, and low dose prednisolone. Because of the unsatisfactory response, pulse dose of methylprednisolone was added. Due to incomplete therapeutic response, intravenous (IV) Tocilizumab was administered for at least 6 months, which resulted in significant improvement, notably in facial morphea. This case report emphasized the importance of accurate and early diagnosis and tailored therapeutic interventions in JS. In patients with LS, attention should be paid to rule out the overlap of systemic and localized types to prevent treatment delay. The presence of Raynaud's phenomenon, telangiectasia, or ANA positivity in a patient with LS should alert the physician to consider the possible coexistence of SSc and LS. Pulse dose of methylprednisolone is the mainstay of treatment in LS. Considering the risk of renal crisis, corticosteroids might be administered cautiously in SSc with frequent monitoring of electrolytes and renal function. Notably, Tocilizumab can be used as a pivotal therapeutic option in managing refractory morphea.