A 12-year-old boy with SCD was presented with fever, periocular pain, and diplopia after returning from Taif, Jeddah. Taif (means “encompassing”) is located in the Hejaz Mountains of Saudi Arabia. It is considered as a high-altitude area because it is 6000 ft above the sea level []. The patient had a previous history of similar attacks that resolved after conservative management at another hospital in the same city few years ago. On admission, the patient looked sick, drowsy, and pale. The temperature of patient was 38.2 °C, heart rate was 115/min, respiratory rate was 25/min, blood pressure was 100/65, and oxygen saturation was 90% on room air. The patient weighted 38 kg. Ocular examination showed right eyelid edema, peri-ocular soft tissue swelling, proptosis, and limitation in elevation of the right eye. On admission, the visual acuity of right eye was 20/30 and left eye was 20/20. Color vision was evaluated using the color plates that came out to be normal. The pupils were equal in size and reactive to light. Swinging light Reflex showed normal reaction of both pupils. There was no afferent pupillary light reflex defect (APD). The intra-ocular pressure was normal in both eyes. Fundus examination revealed normal disc, blood vessels, and macula. A complete systemic evaluation was conducted. The systemic evaluation revealed hemolytic anemia, thrombocytopenia, stable coagulation profile, and negative blood culture. Laboratory results showed hemoglobin level of 89 g/ L, mean cell volume was 84.2 FL, white blood cell count was 24.04X109/L with neutrophils 21.81X109/L, and mean platelets volume 10.30 FL. Serum bilirubin was measured to be 95.5 mmol/L, albumin was 26 g/L, blood urea was 3.8 mmol/L, and serum creatinine was 39 mmol/L. The erythrocyte sedimentation rate was 40 mm/h (normal < 15 mm/h), and C-reactive protein was 8.2 mg/dL (normal < 0.5 mg/dL). The coagulation parameters revealed a prothrombin time (PT) of 14 S (normal 10–12.8), International Normalization Ratio (INR) 1.2 (normal 0.9–1.2) and activated partial prothrombin time (aPTT) 33.4 S (normal 25.3–38.4). Hemoglobin electrophoresis showed HbS 58%, HbA 36%, HbF 2%, and HbA2 4% (consistent with sickle b thalassemia). Urine analysis was normal, and the culture report was negative. Magnetic Resonance Imaging (MRI) of the right orbit demonstrated peri-orbital edema and a mass adjacent to the right orbital wall. This condition was identified as a superior subperiosteal haematoma with evidence of orbital bone and bone marrow abnormal signals consistent with orbital wall infarction. The bone abnormality was further investigated to explore the possibility of the presence of primary or metastatic tumors that are susceptible to bleeding. Therefore, CT-imagery was utilized to explore this area of interest. However, as may be seen from the CT images, there was no evidence of primary or metastatic bone tumors. The patient received intravenous fluids, analgesics, broad spectrum antibiotics, and pulse methylprednisolone immediately. The patient responded well to medical management with complete recovery and was discharged after the condition was stabilized. This case has highlighted the importance of considering orbital wall infarction in the differential diagnosis of orbitopathy among the patients with SCD, along with osteomyelitis and orbital abscess. Careful evaluation, diagnosis, and the prompt initiation of the appropriate supportive care are highly recommended in order to prevent permanent visual loss.