Background: Disruptive amniotic band sequence (DABS) is a sporadic, non-familial disorder with unclear etiology. Diagnosis is based on clinical features because there is currently no reliable laboratory diagnostic tests. Objective: We describe six cases of DABS with severe craniofacial deformations, three with and three without classical constrictive limb deformation. Results: The craniofacial deformities were delimited by peripheral sharply demarcated scarring. Conclusion: When a sharply demarcated linear disruptive craniofacial lesion is observed, DABS should be considered despite the absence of constrictive limb scarring.