The term "juvenile parkinsonism" has been known for over l 00 years. In the majority of cases it is a heterogeneous syndrome. Only the autosomal recessive juvenile parkinsonism was confirmed to be a relatively separate syndrome connected with a mutation in the parkine gene. The early onset (before age 20), dystonic movements, positive response to L-dopa, and early dopainduced dyskinesia are the main phenotypic features of the syndrome. The absence of Lewy's bodies is the main pathological difference between the autosomal recessive juvenile parkinsonism and Parkinson's disease. Recent studies show that parkine mutations are connected not only with autosomal recessive parkinsonism, but may be also found in typical cases of Parkinson's disease with Lewy's body pathology. The diagnosis of juvenile parkinsonism demands its differentiation with many other diseases that may appear in adolescence with such main symptoms as rigidity, tremor and dystonia. In the differential diagnosis first and foremost the L-dopa responsive dystonia and secondary symptomatic parkinsonism should be taken into account.