Timothy syndrome (TS) is a multiorgan disorder with prolonged QTc interval, congenital heart defects, syndactyly, typical facial features and neurodevelopmental problems. Ventricular tachyarrhythmia is the leading cause of death at early age. Classical TS type I results from a recurrent de novo CACNA1C mutation, G406R in exon 8A. An atypical form of TS, type 2, is caused by G406R and G402S mutations in the alternatively spliced exon 8. Only one individual for each exon 8 mutations has been described to date. In contrast to multiorgan disease caused by G406R either in exon 8A or 8, the G402S carrier manifested only an isolated cardiac phenotype with LQT and cardiac arrest. This isolated phenotype was suggested to result from somatic mosaicism. We describe a teenage patient resuscitated from ventricular fibrillation and treated with ICD. She has no other organ manifestations, no syndactyly, normal neurodevelopment and her QTc is <480ms. There is no family history of arrhythmias or sudden deaths. Targeted oligonucleotide-selective sequencing (OS-Seq) of channelopathy genes revealed a de novo substitution, G402S in exon 8 of CACNA1C. This is the third reported case of TS type 2. Direct sequencing of blood and oral mucosa derived DNA showed an identical mutation peak suggesting ubiquitous expression in different tissues. The phenotype of our patient and the previously described patient show an isolated arrhythmia disease with no other clinical manifestations of classical TS.