Celiac disease (CD) is an autoimmune enteropathy resulting from intolerance of an individual genetically predisposed to gluten. It has a large clinical polymorphism ranging from a classic digestive clinical presentation due to the malabsorption syndrome to extra-intestinal symptoms. Among the hematologic abnormalities, venous thromboembolic disease (VTE) has been reported, and they are most often located in the abdomen or lower limbs, but the cerebral localization was exceptionally described. We report a case of CD revealed by cerebral thrombophlebitis. A 44-year-old patient with no medical history and no drug intake, presented with hemiplegia followed by a status epilepticus in a context of apyrexia, initially hospitalized in intensive care. Magnetic imaging resonance displayed a cerebral venous thrombosis of the sigmoid sinus requiring anticoagulant treatment, then transferred to our department for the etiological investigation. On questioning, the patient reported chronic diarrhea and weight loss with no other associated symptoms. The examination revealed an underweight patient with pale conjunctiva, improvement of her deficit symptoms, and no other abnormalities. Laboratory tests noted biological signs of malabsorption. The thrombophilia assessment revealed a protein C deficiency with a slight increase in anticardiolipin antibodies and anti-Beta 2 glycoprotein 1 antibodies. Immunological tests noted positives anti-transglutaminase and IgA anti-endomysium antibodies. Duodenal biopsy demonstrated villous atrophy. After ruling out the other causes of VTE, the diagnosis of cerebral venous thrombosis secondary to CD was retained. Early diagnosis and treatment of CD improves the quality-of-life for patients and may spare them various long-term or even fatal complications.