General anesthesia was needed by children with congenital heart disease (CHD) who underwent cardiaccatheterization procedure and surgery. Pre-anesthetic anxiety in children with CHD can cause significantproblems during induction of anesthesia which leads to negative postoperative outcomes. This studycompared the role of maternal presence during anesthesia induction with midazolam premedication onpre-anesthetic anxiety level in children with CHD. Dr. Cipto Mangunkusumo National Hospital on April toSeptember 2014. Forty-five CHD patients aged 2-5 years old who underwent cardiac invasive procedurewere divided into P group (received midazolam premedication) and M group (had maternal presence duringanesthesia induction). Modified Yale Pre-anxiety Scale (MYPAS) was used for measuring anxiety level ineach patient during preoperative visit, on the time patient entered the procedure room and during induction ofanesthesia. There was no significant difference of MYPAS scores between the two groups in all measurementtimes. The MYPAS score results were non-anxious (median score 23.4) and the highest was at induction ofanesthesia. Inter-rater agreement test between 2 observers was good (k>0.5). In conclusion, there was nosignificant difference between the effect of maternal presence during induction of anesthesia and midazolampremedication on pre-anesthetic anxiety level in children with CHD.