Abstract
Background: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular tumour of the skin which mainly involves the head and neck regions. Case Report: A 41 year old male presented to the ENT outpatient department with a swelling in the anterosuperomedial aspect of right orbital rim since two year. Following a contrast enhanced CT of the Head, complete surgical excision was done and diagnosis of ALHE was confirmed by histopathology. Methodology: We performed a systematic review of the literature following the preferred reporting items for Systematic reviews. Literature searches were conducted in web based search engines using MeSH terms and key words. We found seven publications that fit the inclusion criteria which included case reports and case series. Conclusion: ALHE involving the orbit has been very rarely reported and is a challenging diagnosis. Complete surgical excision is the treatment of choice and recurrence can occur with incomplete removal.