A 2-week-old male infant born at full-term after an uncomplicated pregnancy and labor presented with a congenital cystic scalp nodule. Physical examination revealed a 12 × 11-mm bullous plaque with a red-blue hue on the right vertex scalp surrounded by darker and longer hairs (Figure). A diagnosis of bullous aplasia cutis congenita (ACC) with hair collar sign was made, and magnetic resonance imaging ruled out a central nervous system tract and intracranial extension. Excision at age 6 years revealed skin devoid of hair follicles and adnexal structures with leptomeningeal-like vessels, macrophages, and cerebral cortex, findings that are consistent with ACC with encephalocele.