A 30-year-old woman complained of right-sided epiphora for 2 years. She also reported diplopia on certain gaze positions and felt a hard mass behind the right medial inferior orbital rim. Magnetic resonance imaging studies demonstrated a relatively well-delineated mass in the right inferomedial orbit with minimal ethmoid sinus involvement. Histopathological evaluation following a large incisional biopsy showed massive eosinophilic infiltration and fibrosis with the final diagnosis of eosinophilic angiocentric fibrosis. She was then discovered to have significant peripheral eosinophilia and elevated serum IgE levels and clinical findings of allergic rhinitis and sinusitis. She was treated with systemic fluorocortolon and desloratadin for 4 months. She remained stable without recurrence for 32 months. The patient with this exceptionally rare tumour of the orbit benefited from debulking surgery followed by systemic corticosteroids and antihistaminics.