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Paediatric obsessive compulsive disorder [OCD] is a chronic condition often associated with severe disruptions of family functioning and key developmental milestones. Mean age of onset of juvenile OCD is between 10 and 11 years; however, studies on young children with OCD show that the disorder can manifest itself much earlier. OCD is characterized by a long duration of untreated illness, which, together with an earlier age of onset, has been associated with increased persistence. Although there are disease-specific treatments for OCD, there seems to be difficulty for health professionals to recognize and diagnose the disorder in young children appropriately. This may prolong the interval between help seeking and receiving an adequate diagnosis and treatment. The objective of this review is to give an overview of studies on the clinical presentation, diagnosis and treatment of OCD in very young children.