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Introduction: Eosinophilic fasciitis (EF) is a rare scleroderma-like disease, characterized by acute onset of symmetrical swelling, induration and thickness of the skin. Laboratory findings include peripheral eosinophilia, elevated inflammatory markers and increased gammaglobulemia. Although, the diagnosis is mainly clinically, a full thickness skin/fascia/muscle biopsy remains the gold standard for the definite diagnosis. Unlike systemic sclerosis, visceral involvement, such as pulmonary affection, is rare in EF. Although, few cases of systemic involvement in adult EF have been presented, we report a rare case of juvenile EF associated with pulmonary involvement. Case report: A 12-years old boy presented with symmetrical skin thickening of bilateral upper extremity, back, trunk, neck, face, and scalp of 2-weeks duration. The laboratory tests revealed marked peripheral eosinophilia 61.9% (normal 1–3%) and elevation of the acute phase reactants (erythrocyte sedimentation rate 40 mm/1st hour and C-reactive protein 15 mg/dL). Lung imaging study showed bilateral extensive pulmonary nodules. A full thickness skin/fascia/muscle biopsy revealed an inflammatory infiltration, fibrosis in the fascial and muscle tissues. These findings were concordant with EF. An initial treatment of intravenous (IV) methylprednisolone 30 mg/kg/day for 3 successive days was started followed by oral steroid (2 mg/kg/day) plus methotrexate (20 mg/week). Follow up revealed complete improvement in the skin thickening, pulmonary affection and systemic inflammation. Conclusion: To the best of our knowledge this is the first reported co-existence of pediatric eosinophilic fasciitis with pulmonary affection. Systemic involvement should be screened in EF cases, as it may have consequences in the management and outcome. Keywords: Eosinophilic fasciitis, Eosinophilia, Skin thickness, Pulmonary affection