We report a case of hemoglobin SC disease complicated by fatal pulmonary thromboembolism in a 15 year-old Brazilian boy, submitted to autopsy. This case shows the importance of early diagnosis and treatment of sickle cell disease, which generates systemic involvement in youth and the importance of considering the hypercoagulability in these patients. The autopsy was very instructive, revealing the florid morphologic aspect of sickle cell disease and its complications.