Resumo em português Relata-se o caso de uma paciente com quadro parkisoniano instalado aos 26 anos de idade, 30 anos de evolução, boa resposta à levodopa e desenvolvimento precoce de discinesias induzidas por essa droga. Essas características associadas à ausência de outras manifestações neurológicas ao longo de todo o curso da moléstia sugeriam substrato anátomo-patológico superponível ao da forma clássica da doença de Parkinson. Entretanto, as imagens de ressonância magnét (mais) ica mostraram lesões palidais do tipo "eye-of-the-tiger", semelhantes às observadas na doença de Hallervorden-Spatz. O presente caso ilustra a heterogeneidade patológica dos casos de parkinsonismo de instalação precoce e a possibilidade do encontro desse padrão de lesões palidais em outras doenças degenerativas dos gânglios da base. Resumo em inglês We report the case of a 56-years-old woman patient, born to unrelated parents, who since 26-years-old gradually developed bradykinesia, rigidity, tremor of both hands, and speech and gait difficulties. Her past history was unremarkable. There was no family history of neurologic disease. She was admitted to our Hospital at age 39 and at that time she presented a full parkinsonian syndrome. The following tests were normal or negative: routine blood studies, serum copper, ce (mais) ruloplasmin and cerebrospinal fluid examination. There was not Kayser-Fleicher ring, and fundoscopic examination revealed no abnormalities. Levodopa was introduced and response was good for more than ten years, despite early-onset of dyskinesias (three months after the introduction of the drug). After 30 years under levodopa she still presents a moderate response but with severe fluctuations of the motor performance. Except for slowness of cognition she developed no other neuropsychological impairments, and a recent neurological examination disclosed no abnormalities besides a parkinsonian syndrome. One year ago, a magnetic resonance imaging (MRI) was perfomed and showed bilateral, symmetrical lesions with "eye-of-the-tiger" pattern. This case illustrates the pathological heterogenity of early-onset parkinsonism and suggests the possibility to find the typical MRI lesions seen in Hallervorden-Spatz disease in other degenerative affections involving globus pallidus.