Five cases of congenital ichthyosiform erythroderma are being reported of these three were harleauin babies and two were collodion babies. The harlequin ichthyosis is a rare and dramatic manifestation of severe congenital ichthyosis, the longest reported suruival being 6 wk. The life span of our third case was unsually long, probably because of the intensive care received during the initial weeks. The clinical ana autopsy findings and the recent biochemical aspects are reviewed.