Presented are 2 cases of acute corneal hydrops in keratoconus with Graves' orbitopathy (GO). Two patients (Case 1: female, 54 years old; Case 2: male, 33 years old) with coexisting keratoconus and GO demonstrated typical findings of acute corneal hydrops (ACH) in 1 eye during the active stage of orbitopathy. There was no history of trauma. The ACH healed with scarring after management with a therapeutic contact lens and medical treatment in each patient within 3 months and 5 months, respectively. The development of ACH in keratoconus patients has previously been reported to be associated with male gender, ethnicity, age, eye rubbing, trauma, rapidly progressive disease, atopy, and vernal conjunctivitis. GO involves ocular surface inflammation and fluctuation of intraocular pressure. Active GO can be a risk factor for ACH in keratoconus patients.