Pulmonary capillary haemangiomatosis (PCH) in childhood is a rarity, character ised by the uncontrolled proliferation of pulmonary microvessels which may invad e pulmonary, bronchial and vascular structures, resulting in diffuse alveolar ha emorrhage, manifesting clinically in haemoptysis, dyspnoea and symptoms of pulmo nary hypertension (PH). A 14-year-old boy with some particular features (peric ardial effusion and thrombocytopenia) is presented and 14 paediatric/adolescent cases from the literature are surveyed. The diagnostic problems and difficulties are discussed, including the importance of imaging (high-resolution CT) and hi stopathological studies, with the aim of providing a clear-cut distinction of P CH from other conditions such as primary PH (PPH). The literature data can be re garded as ambiguous: both similarities and relatively sharp distinctions between PCH and PPH are to be found. New developments in the field of genetics are also discussed. The early coexistence of PCH and other (vascular) disorders and asso ciations, involving focal or diffuse, disseminated forms is summarised briefly. Conclusion: The diagnosis of this progressive disorder may lead to effective the rapy. Treatment possibilities include the rapidly evolving field of anti-angiogenic therapy, but at present lung transp lantation is universally accepted as the final definitive treatment for pulmonar y capillary haemangiomatosis. Pulmonary capillary haemangiomatosis (PCH) in childhood is a rarity, character ised by the uncontrolled proliferation of pulmonary microvessels which may invad e pulmonary, bronchial and vascular structures, resulting in diffuse alveolar ha emorrhage, manifesting clinically in haemoptysis, dyspnoea and symptoms of pulmo nary hypertension (PH). A 14-year-old boy with some of the features (pericardial effusion and thrombocytopenia) is presented and 14 pediatric / adolescent cases from the literature are surveyed. The diagnostic problems and difficulties are discussed, including the importance of imaging (high-resolution CT) and hi stopathological studies, with the aim of providing a clear-cut distinction of P CH from other conditions such as primary PH (PPH). The literature data can be re garded as ambiguous: both similarities and relatively sharp distinctions between PCH and PPH are to be found. New developments in the field of genetics are also discussed. The early coexistence of P CH and other (vascular) disorders and asso ciations, involving focal or diffuse, disseminated forms being summarised briefly. Conclusion: The diagnosis of this progressive disorder may lead to effective the rapy. but at present lung transp lantation is universally accepted as the final definitive treatment for pulmonar y capillary haemangiomatosis.