Acute multifocal placoid pigment epitheliopathy ( AMPPE) is a rare inflammatory vasculitis of the choroid. Despite primarily being a disorder of the eye, a number of extra-ophthalmological features have been described in AMPPE and may accompany the visual disturbances. Such patients may be admitted under the care of physicians for evaluation of a systemic illness. We report AMPPE occurring in a 37-year-old man in his thirties admitted with a flu-like illness, erythema nodosum, visual disturbances and raised inflammatory markers. Conditions such as bacterial endocarditis and vasculitis, were considered before a diagnosis of AMPPE was made. The case illustrates how AMPPE can be a diagnostic dilemma, particularly when associated with extra-ophthalmological features. The epidemiology, clinical features, diagnosis and therapy of AMPPE are also discussed.