Introduction: Stroke is an uncommon event in the pediatric population. However, in patients with sickle cell anemia its occurrence is extremely high, with an incidence of 0.61/100 patients/year. Protein C deficiency, by itself, is a thrombogenic factor that increases the risk of stroke in population without sickle cell anemia. The association of sickle cell anemia and protein C deficiency may be related to a higher chance of recurrent stroke. Methods: A descriptive study involving 24 pediatric patients with sickle cell anemia and stroke was conducted in a specialized unit of a Teaching Hospital. Deficiency was considered from below 70% of expected values. Results: Twelve Patients (50%) were female, mean age 11.2 years (4-17). The average age of the first occurrence of stroke was 6.2 years (3-12) and 45% of patients had more than one episode of stroke (mean 1.7 episode/patient). Protein C deficiency was present in 5 (20.8 %) patients. There was significant difference (p < 0.05) in the presence of protein C deficiency among individuals with a stroke episode (7.7%) and patients with two or more episodes (36.4%). Conclusion/discussion: The presence of protein C deficiency in children and adolescents with sickle cell anemia may be related to a higher chance of recurrent stroke. The dosage of protein C in this population may be a useful prevention tool.