We report a family of parkinsonism in which the clinical feature of the constituents varied with the age of onset. The propositus was a 49-year-old woman who had developed tremor and akinesia at the age of 26 years. Levodopa markedly relieved her symptoms. The feet showed pes equinovarus with tonic extension of the great toe. The most characteristic feature was marked diural fluctuation of her symptoms; her gait was nearly normal in the morning, while she showed marked tremor and gait disturbance with parkinsonian posture in the evening. Sleep markedly improved her conditions. Her maternal uncle developed parkinsonism at the age of 60 years, and anti-parkinsonian drugs including levodopa were persistently effective. Neurological examination at the age of 75 years revealed parkinsonian features with rigidity, resting tremor, akinesia and loss of postural reflex, together with severe pes equinovarus and tonic extension of the great toe. There was no diural fluctuation in his symptoms. Several types of dystonia-parkinsonism, either familial or sporadic, have been reported in the literature, but none of them showed the same clinical and genetic features as the present family. The present family may be included in a spectrum of dystonia-parkinsonism syndrome of autosomal-dominant inheritance.