A retrospective study in the ENT departments of the Timone Children's Hospital in Marseille and the Armand Trousseau Hospital in Paris and a review of the literature was performed in order to update knowledge about fourth branchial pouch anomalies. Over the 12-year period studied, a total of six children were treated: three boys and three girls. The lesions were located on the left side in all cases and infection was the most common manifestation. Clinical presentation ranged from suppurative thyroiditis in most cases to stridor in a few newborns. The most useful diagnostic examinations are CT-scan of the neck and endoscopy of the pyriform sinus. The authors emphasize the need for complete surgical resection including the cyst and fistulous tract down to the pyriform sinus.