Beta thalassemia major (also known as Cooley's anemia) is an inherited red blood cell disorder that results in the complete absence or decreased synthesis of the beta globin chains of hemoglobin. Beta thalassemia is among the most common genetic diseases in Morocco and characterized by mild to severe anemia, delay of growth and puberty, enlargement of the spleen, cardiac disease and dysmorphic bone changes. Untreated newborns have a higher risk of death within the first two years of life. Aim: The aim of this study is to evaluate the hematological and nutritional profile in young beta-thalassemia major patient in the Rabat commune. Material and Methods: This retrospective study assessed 104 young beta-thalassemia major patient followed at the division of pediatric hematology-oncology, Rabat children’s hospital, Morocco between October 2014 and 30 June 2015. Patients received regular transfusions every three weeks to maintain hemoglobin levels greater than 10 g/dl. To reduce iron overload, chelation therapy was considered to maintain the serum ferritin levels between 800-1000 ng/mL. Epidemiological, clinical and biological data were collected from medical records and transfusion files of patients. Furthermore, evaluation of the nutritional status and anthropometric measures such as weight-for-age and height-for-ageof subjects were investigated. Results: Data from medical records showed that the average age in our cohort is 11.42 years (the youngest age is 6 and the oldest age is 16). Low hemoglobin count (9.70 g/dl ±1.20) indicateda severe anemia which result in an important microcytosis and hypochromia. Indeed, 39.5% of patient developed leukocytosis, 12.5% neutrophilia, 23% eosinophilia, 9.6% basophilie, 85% monocytopenia, and 28% lymphocytosis. The percentage of subjects presenting stunting and wasting were 39.4%and 19.2%, respectively in patients. Conclusion: This study substantiates that beta thalassemia major young patients require a monitoring from birth, life-long transfusion support, reduction of overloaded iron, and regular nutritional assessment to prevent or reduce the risk of hematological and anthropometric complications.