Atrioventricular block (AVB) is a frequent initial presentation of cardiac sarcoidosis (CS), but dangerous ventricular arrhythmias (VA) can occur. Despite the scarcity of data, guidelines recommend ICD rather than a pacemaker implantation whenever a device is needed. In this study, we aimed to establish predictors for sustained VA in patients with CS presenting with pacing indication due to an AVB. We prospectively enrolled 112 patients with CS. Excluding those with VA, 82 patients remained and were divided into 2 groups: 34 individuals with AVB as initial presentation and 48 with other symptoms as first presentation (OSF). Both groups were compared for clinical characteristics, rates of VA, LVAD implantation, heart transplantation and mortality. During follow-up, VA was detected in 50% in the AVB and 10.4% in the OSF group; P = 0.001. Death, LVAD implantation, heart transplantation occurred in 11.8% in AVB vs. 10.4% in the OSF; P = 0.847. Late gadolinium enhancement (LGE) was equally observed in both groups: 70% vs. 70.5%; P = 0.966, whereas more patients in the AVB group exhibited abnormal PET uptake: 86.2% vs. 54.3%; P = 0.007. In multivariate analysis, AVB (HR 25.15), RV LGE in CMR (HR 7.39) were predictors for VA occurrence, whereas the use of immunosuppressive therapy was associated with less VA (HR 4.3). Patients with CS presenting with AVB have a high risk of sustained VA. Although immunosuppressive drugs may reduce the occurrence of VA, ICD implantation is reasonable, especially in case of right ventricular LGE.