A review of the current state of research in the genetics of obsessive-compulsive disorder (OCD) is presented. OCD is a neuropsychiatric condition that affects 1-2% of the population and often has an early age at onset of symptoms. OCD has been shown to be familial, and a major gene effect has been reported. However, phenotypic and genetic heterogeneity of OCD poses multiple challenges for locating susceptibility genes. Strategies such as the use of phenotypic subtyping (using tic disorders or other anxiety disorders) and endophenotyping based on brain mechanisms underlying OCD (functional brain imaging and neuropsychological measures) may open ways to understand the genetic components of OCD. Using child probands and extended families for linkage an association studies is another venue to obtain greater informative families for genetic studies. A better understanding of environmental triggers, OCD subtypes and OCD pathophysiology will lead to locating genes that confer risk to OCD.