Primary pulmonary hemosiderosis (PPH) is a rare, but potentially lethal disease. There are various theories explaining the origin (genetic, autoimmune, allergic, environmental, metabolic), but precise mechanism is still unknown. PPH is characterized by repeated episodes of inrtraalveolar bleeding with subsequent abnormal accumulation of iron in the form of hemosiderin in lung macrophages, roughening of alveolar basal membranes and interstitial fibrosis. One of the subtypes of PPH is the Heiner syndrome (HS), described for the first time 40 years ago. It is a lung disease caused by allergy to the proteins of cow’s milk (CMP). In addition to the classical triad of PPH symptoms (hemoptisis, sideropenic anemia, diffusion lung infiltrates on X-ray picture), there is a typical finding of high titres of precipitins against CMP and improvement of clinical condition after exclusion of cow’s milk from the diet. In contrast to other types of PPH, there is better prognosis for HS, and long-term immunosuppressive or corticoid therapy is usually not necessary. Although the disease is relatively rare, it should be kept in mind in differential diagnosis of chronic pulmonary diseases of uncertain etiology in children with obvious symptoms of dietary allergy.

        Key words: pulmonary hemosiderosis, cow’s milk allergy, respiratory signs, precipitins, milk elimination, child