Paraoesophageal hernia (PH) in children is a rare entity, and most reported cases refer to adult patients. Its etiology is not precisely known, but the hypothesis of its congenital origin is widely accepted. Similarly to other congenital diaphragmatic defects, PH follows a sporadic pattern of incidence in most cases. Familial occurrence of sliding hiatal herniae has been reported in more than 20 cases, but only one family with two members affected by PH was described in the literature. We present two pairs of siblings with the paraoesophageal type of hiatus hernia and discuss the clinical presentation of this anomaly in children.