Arrhythmogenic right ventricular dysplasia is a rare but not exceptional inherited cardiomyopathy characterized by fibrofatty replacement of the myocardium of the right ventricular which could lead to serious arrhythmia and sudden death. Only a few cases of pregnancies with ARVD have been reported. The aim of this case presentation is to describe the clinical characteristics and anesthetics specificities in management of this disease in pregnancy and in delivery. We report the case of a young woman aged 28 years old with a past history of ARVD treated by medical treatment with radiofrequency ablation. This patient was pregnant and it was scheduled for cesarean section delivery. Preoperative evaluation showed a well tolerated pregnancy inspite of the severity of the ARVD. It was a severe form of ARVD because of RV dilation, the spread of the disease to LV and the history of ventricular tachycardia during an attempted of ablation by radio frequency. The treatment received by the patient was kept until the day of surgery. The act took place under general anesthesia. The postoperative period was uneventful and morphine titration was used for pain relief. The patient exits the hospital 3 days after delivery and breastfeeding was forbidden. We should pay attention on this disease witch is not yet well known and witch is highly risky in the peri-partum period. Even if pregnancy may be tolerated in moderate forms of ARVD, conception and delivery sould be discouraged especially in severe forms.