We report herein a case of fetal complete atrioventricular block accompanied with endocardial cushion defect, successfully diagnosed and treated, in utero, with transplacental digitalization. A 23-year-old Japanese woman, at 20 weeks of gestation, was referred to the Maternity and Perinatal Care Unit of Kyushu University Hospital because of fetal continuous bradycardia. B-mode scanning and dual M-mode echocardiography revealed that the fetus had complete atrioventricular block with endocardial cushion defect with a ventricular rate of 60 beats per minute. At 23 weeks of gestation, it was found that the fractional shortenings (FSs) in both ventricles and the ventricular rate had decreased, with an increase in pericardial effusion. Thus, we diagnosed the fetus as having cardiac failure. Transplacental digoxin treatment was started and continued for 10 weeks, after which fetal pericardial effusion, as well as FSs ameliorated. The pregnancy was interrupted by cesarean section at 33 weeks of gestation due to a decrease in FSs with an accumulation of fetal ascites. A 1780g female infant was delivered and a pacemaker was implanted surgically, immediately after birth. She is alive and well at the time of writing.