Obsessive-compulsive disorder (OCD) has been described in preschool children as young as 2–3 years old. A preschool age onset of OCD has unique diagnostic and therapeutic challenges. In this article, the authors review published literature on preschool onset OCD and present data on preschool-onset OCD (age of onset ≤ 5 years) from clinical records at a tertiary care child and adolescent psychiatry center in India. Literature suggests that OCD that starts this early is phenomenologically similar to OCD in older individuals; however, it has very high rates of comorbidity and a family history of OCD. There is a paucity of data on course, treatment, and long-term outcome in this group. At their center, the authors found a 3% prevalence of preschool-onset OCD, with a male predominance (69%) and fairly high comorbidity rates (62%). Qualitative review highlighted delay in treatment seeking, poor follow-up rates, frequent use of pharmacological treatment, and a high remission rate in those treated adequately.