Abstract
Congenital atrichia a rare form of alopecia in children, presents with complete absence or sparse hair over the scalp and body, while the teeth, nails, and sweat glands are normal. Uncommonly, this hair abnormality can be associated with any systemic or cutaneous abnormalities. We report three cases of congenital atrichia with parental nonconsanguinity and central nervous associations such as developmental delay,spastic quadriparesis, failure to thrive, myoclonic seizures, and attention deficit hyperactivity disorder. The presence of papular lesions in two among the three children further supplemented our case report. Genetic analysis and counseling regarding the constancy of the condition will be the foremost important part of management.