Background: Sickle cell disease (SCD) is considered as one of the major types of anemia in Sudan, especially the western part of the country. We studied clinical and hematological findings to obtain an initial baseline data on sickle cell anemia in children in North Kordofan in general. Materials and Methods: Total 75 children were enrolled in the study prospectively using a questionnaire. Clinical data, as well as hematological parameters of full blood count (FBC) for each patient, were carefully obtained. Results: About 80% of the children were initially diagnosed at age less than 5 years. The study showed high frequencies of sickle cell disease among Falatah (13.3% vs 16%) and Jawama (12% vs 13.3%) for fathers and mothers, respectively. Total 80% of children’s parents were relatives or from the same tribe. The most frequent clinical presentations were fever (84%), pallor (79%), and bone pain (62%). While common clinical findings were hepatomegaly (44%), joint swelling (35%), and bone swelling (31%). The overall mean hemoglobin concentration was 8.0 ± 2.4 g/dl, packed cell volume was 24.5 ± 7.0 percent, total white blood cells count was 14257 ± 7302 cells/ μL, platelets count was 470821 ± 162245 cells/ μL and reticulocytes count was 12.1 ± 9.8 percent. Conclusion: Epidemiological surveys are needed to determine exactly the magnitude of this problem at least in the region as well as designing a strategic plan to address urgent and long-term interventions in control of sickle cell disease.