Hereditary multiple intestinal atresia is an extremely rare subgroup of intestinal atresias, with an autosomal recessive mode of inheritance. It presents a unique combination of clinical, radiological and pathological findings and is secondary to a malformative process taking place early in intrauterine life and effecting the whole gastrointestinal tract. Multiple intestinal atresia is mainly characterized by multiple and widespread atresias extending mostly from pylorus to rectum, intraluminal calcifications on plain abdominal roentgenogram, and an invariably fatal outcome. Cystic dilatation of the bile ducts can be present in cases with both complete pyloric and duodenal or proximal jejunal atresia.