Sickle cell disease (SCD) is a collective term for a number of genetic disorders in which hemoglobin is structurally abnormal, resulting in the episodic formation of sickle-shaped red blood cells (RBCs) and a wide range of clinical manifestations.Aims: To identify the common infections in children with sickle cell disease in KSA regarding its prevalence, infection sites, as well as type of pathogens.Methods and Material: study conducted on 100 sickle cell disease patients who attended emergency department, King Abdulla Hospital, KSA. All children with SCD presenting with symptoms and signs suggestive of infections or acute complications of sickle cell anaemia.The identification of the infecting organism was done by culture studies.Results: Patients' age ranged from 2 to 18 years old with a mean of 13.12±4.85 (SD); 68 patients' were females while 32 were males and 95% of patients had positive family history of consanguinity. Nine cases had positive blood culture (9%) and urine culture 12 cases (12%). Out of the 21 positive microbial infection cases; 12 cases were diagnosed as Urinary Tract Infection, 4 cases as Osteomyelitis, 2 cases as Fever for investigation and 3 cases as Sepsis. All cases of pneumonia were diagnosed clinically .Two of the Osteomyelitis cases were Staphylococcus species and other 2were Salmonella. In all cases diagnosed as Fever for investigation and Sepsis, the organism was Staphylococcus species.Conclusions: Urinary tract infection was the most common infectionE. Coli was the most common organism isolated followed by Staphylococcus species, Klebsiella Pneumonia and Salmonella.