Less is known about pregnancy in women with arrhythmogenic right ventricular cardiomyopathy (ARVC). From April 1995 to May 2018, 157 women with ARVC were retrospectively enrolled. Data on pregnancy and cardiac outcomes were analyzed. There were 224 pregnancies in 120 patients including 30 (13.4%) spontaneous and 2 (0.9%) medical abortions, 12 cardiac adverse events were recorded including new onset frequent premature ventricular contractions (PVC) in 3 (2.5%) patients, previous PVC numbers increased more than 100% in 5 (4.2%), syncope in 2 (1.7%), sustained ventricular tachycardia and heart failure required hospitalization each in one patient (0.8%). Women with cardiac events showed lower left ventricular ejection fraction (LVEF) (50.3 ± 2.7 vs 60.0 ± 7.3; p = 0.004). No significant change in cardiac structure and function was found at 1 year follow-up postpartum. At a median follow-up of 8 (1 to 32) years, 36 (22.9%) women died. Earlier symptom onset age (hazard ratio 1.046; 95% confidence interval 1.017 to 1.075; p = 0.002) and decreased LVEF (hazard ratio 1.127; 95% confidence interval 1.001 to 1.154; p = 0.041) increased the risk of all-cause mortality, pregnancy had no negative influence on survival. In all the 192 offsprings (mean age 26.3 ± 13.5 years), 2 died of sudden death, no definite ARVC was found. Pregnancy seemed to be acceptable in ARVC, decreased LVEF increased the risk of pregnancy and was associated with poorer long-term survival.