Uniportal or single-incision video-assisted thoracic surgery (VATS) has been performed successfully in adult patients with different intrathoracic pathologies for years. However, no report in uniportal/single-incision VATS in pediatric patients in the English literature has been published up to date. This may be explained by the limited number of patients and the difficulties in working in very narrow thoracic cavity of babies and children. For these reasons, all the published cases of VATS for extralobar sequestration in infants or children were performed through the three-port approach. We report herein a case of single-incision VATS in a child for the resection of an extralobar sequestration.