We describe an infant with aplasia cutis congenita of the scalp complicated by sagittal sinus hemorrhage. The defect was successfully managed by primary closure with scalp flaps. The literature on aplasia cutis congenita is reviewed, including etiology, genetic transmission, associated anomalies, and options for management. The potentially fatal outcome highlights the importance of early surgical coverage of the exposed superior sagittal sinus to avoid life-threatening hemorrhage and other complications.
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