Several studies suggest that patients with giant cell arteritis (GCA) presenting with isolated polymyalgia rheumatica (PMR) with no cranial symptoms are at low risk of suffering GCA-related ischemic events. However, the issue remains controversial. In the current study we assessed the development of ischemic events in a large series of GCA patients who suffered from apparently isolated PMR during the main course of their disease. One hundred GCA patients presenting with PMR only for at least 2 months were selected from among 347 individuals with biopsy-proven GCA. Clinical manifestations and their chronologic appearance before diagnosis were recorded. Seventy-three patients presented with isolated PMR for a median of 8 months (range, 2 mo-5 yr) and later developed cranial symptoms for a median of 3 weeks (range, 0 wk-1 yr), which eventually led to GCA diagnosis (Group 1). The remaining 27 patients, after presenting a self-limiting course of dismissed mild cranial symptoms lasting for a median of 2 weeks (range, 1 wk-4 mo), developed PMR, which was their chief complaint for a median of 3 months (range, 2 mo-1.5 yr) and the reason for medical evaluation (Group 2). Twenty (27.4%) patients in Group 1 suffered disease-related ischemic complications at the time of diagnosis. No patient in Group 2 developed ischemic events. Patients with GCA presenting with apparently isolated PMR are not a benign subset and have a significant risk of developing ischemic complications. Among them, the only patients who appear to be at low risk of developing ischemic events are those in whom a self-limiting episode of cranial symptoms can be recorded.