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Primary Pleural Inflammatory Pseudotumor in a Child. BACKGROUND: Inflammatory pseudo tumor, a rare non-neoplastic lesion, commonly presents as slow growing solid lesion in the lung, but many extra-pulmonary locations have been described. CASE CHARACTERISTICS: A 4-year-old girl who presented with respiratory distress due to massive pleural effusion. Computed tomography revealed large hypodense non-enhancing lesion in the left hemi thorax. Surgical exploration revealed large semisolid pleural collection filled with gelatinous material with normal underlying lung. OUTCOME: Histopathology revealed spindle shaped cells with abundant myxoid stroma. Child recovered after surgery and was asymptomatic at 5 years follow-up. MESSAGE: Primary pleural inflammatory pseudotumor may be a rare cause of pleural effusion in a child.