Key Points: Hermansky–Pudlak Syndrome

Hermansky–Pudlak syndrome is a rare autosomal recessive platelet delta storage pool disorder associated with systemic manifestations. Available data suggest low rates of antenatal bleeding complications in pregnancy, although published experience remains limited. Data on the guiding mode of delivery is sparse. Regional anesthesia is generally avoided, and instrumental delivery is typically minimized due to bleeding risk. Hemostatic management during delivery commonly includes desmopressin and or platelet transfusion, recognizing variable responses and limited evidence.