BackgroundAlthough sickle cell disease is an important public health problem in Brazil, there is a gap in the literature on the level of knowledge of primary health care professionals about the treatment and management of sickle cell disease. Therefore, this study aimed to evaluate the level of knowledge about sickle cell disease of physicians and nurses who work in the Family Health Program in a region of Brazil with a high prevalence of this disease.MethodsThis is a descriptive, cross-sectional study conducted at the municipality of Montes Claros, in the north of Minas Gerais, Brazil. Study participants included 96 physicians and nurses who work at the Family Health Program in an urban area of the city. Data was collected using an original, partially tested questionnaire based on health care check points for children with sickle cell disease established in educational protocols from the State Health Secretary of Minas Gerais and the Ministry of Health. The structured questionnaire contained 47 questions addressing three axes: epidemiology (8 questions); clinical manifestations (13 questions); and management of children with sickle cell disease (26 questions). Knowledge was measured through mean correct responses to proposed questions. Ethical principles were respected and this project was approved by the Committee of Ethics in Research.Results59.4% (57) of the study participants were nurses and 40.6% (39) were physicians. The median length of training and median length of service in primary health care were 4.3 (2.8-8.0) years and 4.0 (2.0-7.1) years, respectively. The mean performance in knowledge tests was < 75%, with 5.7/8 (SD = 1.4) for the "epidemiology" questions; 8.6/13 (SD = 2.2) for "clinical manifestations"; and 17.0/26 (SD = 2.9) for "management of children with sickle cell disease" questions; resulting in a mean total of 31.4/47 (SD = 5.10) correct responses. A statistically significant association was found between the number of correct responses and family health care qualifications (p = 0.015).ConclusionThere is an urgent need to improve primary health care professional training in the care of children with sickle cell disease.