ABSTRACT Background: Beta thalassemia major is a disorder characterized by defective hemoglobin production and excessive destruction of red blood cells. This life-threatening illnesses suffered by children can lead to parental stress and depression. How to cope with stress coping strategies may be used. Coping strategy is a way by individuals to deal with and anticipate situations and conditions that are pressed or threatened both physically and psychologically. Therefore, parents who have children with beta thalassemia major require more attention. This study aimed to determine the relationship between stress and coping strategies of parents who have children with beta thalassemia major in the city of Banda Aceh. Methods: Cross sectional study conducted in 71 mothers of children with beta thalassemia major with total sampling method. The research was conducted on 14th December 2012 to 14th February 2013 in the Space Center Thalassemia dr. Zainoel Abidin Banda Aceh. Stress was measured with a questionnaire Parental Stress Scale (PSS) and the coping strategies measured by the Brief Cope questionnaire. Definitive diagnosis of beta thalassemia major in children was got from the medical record. Results: The results of this study showed as much as 45,1% of respondents were aged 30-39 years. Respondents who generally work as housewives 52.1%. education level of respondents generally basic education 45%. The majority of respondents got moderate stress level as mush as 46.5% and the coping strategies was in the high category about 60.6%. From the analysis using chi-square test, it was obtained that p-value = 0.042 (alpha