BACKGROUND The Churg-Strauss syndrome (CSS) is a rare multiple organ disease, that belongs to the group of systemic granulomatous vasculitis. The initial symptoms are often bronchial asthma and allergic rhinitis; later in the course of the disease, the patients exhibit lung, heart and kidney manifestations. METHODS We report on a thirty-one year old female patient who initially presented with asthma and polypous sinusitis. Three years later, after undergoing endonasal sinus surgery three times, she was diagnosed with CSS. Within a review of the literature, we discuss symptomatology, differential diagnosis, and therapy of the CSS. RESULTS Worldwide about 200 cases are published. The patient presented in this case report is now in a good health condition after a four-year therapy with steroids. The patient is free of sinusitis symptoms without the need of renewed surgical intervention. CONCLUSIONS CSS should be considered in the differential diagnosis if bronchial asthma and resistant polypous sinusitis occur coincidentally. Often it is very difficult to diagnose CSS. Frequently it is only diagnosed after several years. Therapy of choice is the treatment with steroids, sometimes supplemented by cytotoxical drugs.