Aplasia cutis congenita (ACC) is a rare disease with an unclear pathogenic mechanism. ACC has been suggested to result from the disrupted development or degeneration of skin in the uterus. This study describes two cases that may have underlying pathogenic cause that have not been previously reported. Two neonates who were admitted to the neonatal intensive care unit due to "skin lesions on the limbs" without other deformities or complications were diagnosed with type VII ACC by dermatologist. The mothers showed positivity for hepatitis B virus (HBV) surface antigen and elevated level of HBV DNA copies, which may be related to ACC. But this association could be a coincidence. Both neonates were treated with antibacterial dressings and achieved satisfactory healing.