Introduction: Sweet syndrome, or acute febrile neutrophilic dermatosis is a rare inflammatory disorder involves multiple organs, characterized by fever, high inflammatory markers and distinct skin rash. Pulmonary involvement is extremely rare in paediatric population and literature was not found for paediatric patients. Case report: One year and four months old boy presented with fever, cough and respiratorydistress for three days duration with generalized erythematous, plaques like lesions. The skin biopsy revealed neutrophilic infiltrates in the dermis without vasculitis. The diagnosis of Sweet syndrome with pulmonary infiltrates was made and started on oral prednisolone and dapsone. Evaluated for secondary causes (haematological malignancy , Inflammatory bowel disease, Immunodeficiency) and excluded. Genetic studies didn’t reveal pathological variants for Sweet syndrome. High resolution computed tomography of chest showed centrilobular nodules in bilateral apical segments. He was the first product of healthy nonconsanguineous parents with an uncomplicated antenatal period and developed multiple episodes of generalized maculopapular rash with fever and high inflammatory markers. After one year he had multiple exacerbations of pulmonary disease which responded to high dose steroids. He was started on Tocilizumab to control disease activity and alleviate steroid toxicity . Initially he responded, but developed severe sepsis and respiratory failure following subsequent doses of Tocilizumab and succumbed to death at the age of two years and ten months. Conclusions: Sweet syndrome with pulmonary involvement is extremely rare in paediatric age group with significant mortality and morbidity . Steroids are the mainstay of treatment. Biologics can be used in refractory cases with caution.