A 62-year-old Chinese woman, with a history of immunoglobulin A nephropathy, presented with an ulcerative conjunctival lesion and anterior orbital inflammation. No infective foci were found, and her symptoms were not alleviated by antibiotic treatment or drainage of the lesion. She was diagnosed with idiopathic orbital inflammatory disease, also known as orbital pseudotumor, which completely resolved with oral corticosteroid treatment. To the best of our knowledge, this is the first documented case of idiopathic orbital inflammatory disease that initially presented with an ulcerative conjunctival lesion. We illustrate our stepwise diagnostic approach and management of the disease.