We report a 24-year-old man with Vogt-Koyanagi-Harada (VKH) syndrome who developed a macular hole (MH) during the acute inflammatory stage. Spontaneous resolution was unlikely because of the MH dimensions and absence of vitreous adherence. The patient underwent pars plana vitrectomy (PPV) and internal limiting membrane peeling during the acute stage followed by retinopexy with octafluoropropane injection and prone positioning for 5 days. The MH closed and the best-corrected visual acuity (BCVA) improved from 20/400 to 20/40. Prompt surgical intervention may be an alternative for treating MHs and obtaining visual recovery in special cases even in the acute inflammatory stage.