Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare clinicopathologic entity that shares both clinical and histopathological features with Kimura disease. Although they were once considered as different stages of the same disease, they are now known to represent separate entities. ALHE is a condition that causes swellings in the head and neck region affecting muscular arteries and is benign in nature. Orbital involvement is unusual. Diagnosis of ALHE is frequently confused with malignant tumour until biopsy is done. We present here a young female with unilateral, painless and slowly progressive swelling on the inner and upper aspect of her left orbit, who was diagnosed as a case of ALHE with orbital involvement on the basis of CT scan and excisional biopsy. Regular follow up was performed and no evidence of recurrence was found.