Background - Aplasia cutis congenita is a congenital absence of skin in new born. It can be anywhere over body. Aplasia cutis congenita is a rare congenital disorder of skin. We are presenting a case of aplasia cutis congeni Case report – ta in female child associated with pyomeningitis. Conclusion - Aplasia cutis congenita, its rarity and unknown etiology is the reason of reporting. It is rarely reported a case of Aplasia cutis congenita with association of pyomeningitis.