Sclerosing haemangioma of the lung (SHL) should be recognised as a distinct clinicopathological entity. It is a benign neoplasm, probably of epithelial origin. Clinically, the tumour is asymptomatic and shows a striking preponderance in middle-aged women. SHL is often detected incidentally, as a round, well-defined homogeneous mass on routine chest radiograms. The diagnosis is based on pathohistologic examination of the biopsy material, therapy is surgical, and prognosis is excellent. We report a case of a 4-year-old boy with SHL, which is extremely rare in childhood.