A Spanish family with the DTI variant sensitive to L-Dopa is presented with three patients, 2 first cousins and an aunt being described. The dystonic beginning of the disease during infancy is of note in the former two patients as is the later initiation of parkinsonism in the third patient. Marked improvement was observed in all of the patients with L-Dopa treatment. Some peculiarities of the cases are commented upon and the discussion includes analysis of the nosological aspects, relation with juvenile parkinsonism and Parkinson's disease. Finally, reference is made to the different hypersensitivity for presenting choreic dyskinesia which the patients had.