Acute lung injury (ALI), including acute respiratory distress syndrome (ARDS), is a critical condition consisting of acute hypoxaemic respiratory failure with bilateral pulmonary infiltrates of non-cardiogenic origin. Occasionally it develops in patients with acute or subacute central nervous system pathologies, such as increased intracranial pressure and brain stem lesions. The exact epidemiology of neurogenic pulmonary oedema is unknown. However, due to non-specific clinical manifestation it often remains undiagnosed. Its pathogenesis probably involves overactivation of the sympathetic autonomic system with pulmonary hypertension and increased vessel permeability. We present a case of successfully managed ARDS in a previously healthy patient with newly diagnosed and symptomatic haemangioblastoma of the medulla oblongata. In this context we also review current knowledge on the aetiopathogenesis, diagnostic criteria and treatment for neurogenic ARDS.