We identified rare inherited CNVs at positions congruent with curated DECIPHER syndromic loci, and overlapping genes associated with other neurodevelopmental disorders, FMRP targets, or BTBD9. In 10/174 (5.7 %) of our trios, we identified an inherited genetic lesion that we propose might have contributed to the patient’s OCD phenotype. This evidence concerns the gene BTBD9 and neurodevelopmental disorder.