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We report four patients with acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with atypical features which were unilaterality, serous detachment of the neurosensory retina, retinal vasculitis, and papillitis. All patients responded well to oral corticosteroids. We suggest that systemic corticosteroids could be useful in selected cases of APMPPE with atypical clinical features.