Background: The reported incidence of venous thromboembolism in sickle cell disease patients is approximately 12% before the age of 40, accompanied by an additional 5-10% incidence of cerebral venous sinus thrombosis. Tentorial sinuses are venous structures in the posterior cranial fossa, draining the occipital and temporal lobes and the superior part of the cerebellum. Here, we describe a rare case of lateral tentorial sinus thrombosis in a patient with sickle cell disease. Methods: Case report and review of the available literature Results: A 26-year-old woman with sickle cell disease presented with a 2-day history of headache, nausea, and vomiting. Neurological exam was normal. CT brain revealed hyperdensity in the right occipital region and subtle area of hypodensity in the right medial occipital lobe. MRI/MRV brain showed right lateral tentorial sinus and occipitobasal vein thrombosis in addition to right medial occipital lobe venous infarct. The patient was started on therapeutic heparin initially, then transitioned to apixaban. Hemoglobin electrophoresis revealed a HbS fraction of 80.6% and HbF 16.6%. The patient received an exchange transfusion and was discharged home in stable condition. Conclusions: In patients with symptoms concerning for cerebral venous thrombosis, attention is usually directed to the traditional dural venous sinuses and cortical veins, while other venous structures can be overlooked. Although, in this particular case, it was clear on imaging, tentorial sinus thrombosis can easily be missed if not associated with brain edema or venous infarct.