BackgroundChildren with sickle cell disease (SCD) frequently present with acute pain. The abdomen, a common site of acute SCD‐related pain, may be present in a variety of gastrointestinal (GI) pathologies. Limited data exist on prevalence and workup of abdominal pain in patients with SCD during acute pain events.ObjectivesDetermine prevalence of GI symptoms, GI‐specific evaluation and risks of hospitalization in children with SCD presenting to the emergency department (ED) or hospitalized with abdominal pain.MethodsRetrospective study of children less than 21 years presenting to the ED or hospitalized with pain in our center over 2 years. Descriptive statistics were used to report clinical characteristics, frequency of GI symptoms, workup by age (<5 vs. ≥5 years), and genotype (sickle cell anemia [SCA] vs. non‐SCA). Logistic regression models were used to identify risks associated with hospitalization.ResultsA total of 1279 encounters in 378 patients were analyzed; 23% (n = 291) encounters were associated with abdominal pain. More abdominal pain‐associated hospitalizations occurred in older children, SCA, children with lower mean hemoglobin (8.7 ± 1.9 vs. 9.6 ± 1.6 g/dL, p < .001) and higher mean white blood cell (WBC) count (14.9 ± 6.6 vs. 13.2 ± 5.3 × 103/μL, p = .02). We identified that less than 50% of patients presenting to the ED with abdominal pain received a GI‐specific evaluation.ConclusionChildren with SCD frequently present with abdominal pain and other GI symptoms, with limited GI evaluations performed. GI‐specific evaluation may increase diagnosis of GI pathologies, rule out GI pathologies, and contribute to the limited knowledge of the abdomen as a primary site of SCD pain.