We report a case of lacrimal gland pseudotumor as the presenting sign of ulcerative colitis. A 25-year-old woman presented with a right upper eyelid swelling and pain. Intravenous administration of prednisolone was initiated on suspicion of lacrimal gland inflammation (pseudotumor). Although the treatment markedly reduced her ocular symptoms, she developed lower abdominal cramping and diarrhea with the 5 mg/day of oral prednisolone. Sigmoid colonoscopy and colon biopsy led to make a diagnosis of ulcerative colitis. Ulcerative colitis improved significantly with increased dose of steroid and additive mesalazine therapy. Ulcerative colitis should be included in the differential diagnosis of lacrimal gland pseudotumor.