A 47-year-old male, a known case of Bernard–Soulier syndrome diagnosed on the basis of thrombocytopenia with giant platelets on complete blood count and peripheral blood smear, absent ristocetin-induced platelet aggregation uncorrected by normal plasma, and reduced expression of the platelet glycoprotein GPIb–IX–V complex on flow cytometry, presented with acute neurological symptoms. Computed tomography of the brain revealed intracranial and subdural hemorrhage. On admission, a complete blood count showed severe thrombocytopenia with a platelet count of 30 × 10⁹/L and hemoglobin of 10.2 g/dL. Peripheral blood smear demonstrated large and giant platelets, some hypogranulated, consistent with Bernard–Soulier syndrome. During hospitalization, the patient received multiple single-donor platelet transfusions as part of the management of this life-threatening bleeding episode.

This case highlights a severe hemorrhagic complication of Bernard–Soulier syndrome, an inherited platelet adhesion disorder caused by defects in the glycoprotein Ib–IX–V complex, predisposing affected individuals to spontaneous or trauma-related bleeding, including intracranial hemorrhage.