Acute haemorrhagic oedema of infancy (AHOI) is not a clinically well-recognized disease. We present the case of a 10-month-old girl with AHOI, and compare the clinical, histopathological and immunohistological features of this acute purely cutaneous leukocytoclastic vasculitis with those of the more frequent Henoch-Schonlein purpura. AHOI should be regarded as a separate clinicopathological entity for correct prediction of prognosis and prevention of unnecessary treatment.