Isolated acquired macroglossia of tongue rarely reported. It occurs due to causes like hereditary angioedema, localized angioedema, etc., Here we describe an 8-year-old boy developing life threatening localized angioedema of tongue due to phenytoin without any association with drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome or pseudolymphoma encountered in rural medical college. Anticonvulsants, that is, phenytoin induced this isolated peculiar complication, which was not described before.