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, reduced expression of the platelet glycoprotein GPIb–IX–V complex on flow cytometry, and genetic testing revealing a pathogenic variant in the GP1BB gene (GP1BB: NM_000407: exon 2: c.423C>A: p.C141X), presented with acute neurological symptoms. Computed tomography of the brain revealed intracranial and subdural hemorrhage. On admission, 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.