One of the objectives of this large scale cross-sectional study of school children of the Addis Ababa city was to assess the status of rheumatic heart disease (RHD) prophylaxis among rheumatic heart disease patients identified during the survey. Awareness about the presence of the illness in those affected and reasons for poor coverage, when detected, were also assessed. Sixty of the 9388 school children surveyed were found to have rheumatic heart disease. On interviewing parents of the children with rheumatic heart disease, ten acknowledged being informed of their children's cardiac illness. Of these parents, 15% (or 9/60) had some idea that their children had heart disease related to some form of infection. However, only two of the nine (22%) children whose parents had some idea about their disease were on regular monthly benzathine penicillin prophylaxis in the previous 12 months preceding the interview. Three (33%) of the nine children had six or fewer injections in the 12 months preceding the interview. The remaining 4 parents (44%) reported that their children took treatment that included injections only at the time of initial diagnosis several years earlier and had not had any follow up since then. Their reasons for not seeking medical care for their children included lack of information on prophylaxis, inability to pay for the treatment and distance of the health facilities. The lack of awareness and the extremely low rate of regular prophylaxis, therefore, highlight the need for an urgent control programme that takes active case detection, treatment access and health education into consideration.