An 11-day-old boy born to nonconsanguineous Vietnamese parents presented to our pediatric dermatology outpatient department with a congenital linear scalp and face lesion. This soft, skin-colored plaque following the lines of Blaschko was devoid of terminal hair growth and extended from the left upper eyelid up to the ipsilateral vertex. At the vertex were 3 round, elevated, bald, bullous patches and 2 round scars and hair collars (Figure 1). On the upper eyelid, 2 soft, skin-colored papules were seen. Based on the clinical evaluation, encephalocraniocutaneous lipomatosis (ECCL) was suspected.