We present the case of a 10-year-old boy, Sam, with congenital deafness and Gilles de la Tourette Syndrome (GTS). GTS is characterised by multiple motor tics and one or more vocal tics that wax and wane. Due to his deafness Sam never developed vocal language but instead used sign language from the age of four. His tic disorder rapidly accelerated from the age of seven over a six-month period and soon sign language was incorporated into tics as complex "vocal" tics. Bursting out "words" in sign language would also occur in front of people unfamiliar with sign language and often with an obscene content although this was not evident to someone not trained in sign language. To our knowledge this is the first reported case of a congenital deaf child with GTS. The case presented here supports previously published work that the intentional share of the tics in GTS is very small. This case also questions former theories on which regions and circuits of the brain are involved in GTS.