Harlequin fetus is a rare clinical entity, and survival of affected infants beyond the first year of life is uncommon. Management involves intensive care of the skin and eyes, close monitoring of fluid and electrolyte status, constant support and counselling of parents, and surveillance against infection and side-effects of medication. A well-coordinated multidisciplinary approach can prolong survival beyond the neonatal period. We report our experiences in the management and follow-up of two successive harlequin siblings.