Pulmonary Sequestration and Pterygium Colli Syndrome

A rare case of a male pterygium colli syndrome associated with an extralobar pulmonary sequestration is reported. The patient had webbed neck, low-set ears, low-set hair line, aplasia of the left kidney and hypoplastic mesenterial vessels referring to Turner's syndrome, but a normal XY sex chromosome constitution. The pulmonary sequestration was resected and the patient made an uneventful recovery.