BACKGROUND: Williams-Beuren syndrome (WBS) is a developmental disorder of unknown etiology. The classical features include: 1) a supravalvular aortic stenosis, 2) "elfin" facies and 3) mental deficiency. Ocular findings can be irisabnormalities (stellate, pattern), tortuositas vasorum and, though not essential for diagnosis, strabismus is commonly found. Three cases of WBS with convergent squint which underwent surgical treatment are presented. Biopsy and histological studies were performed in two cases. MATERIAL AND METHODS: Strabismus onset was diagnosed before the second year of life in all three. Pre-operative esotropia (ET) was 45, 40 and 62 delta. Age at operation was respectively 52,67 and 87 months. Surgical treatment consisted in recess-resect procedures on horizontal muscles. Biopsy was performed in two cases. RESULTS: Three months after surgery, angular measurements were respectively: exotropia (XT) 6 delta, ET 2 delta, ET 10 delta. Anatomopathological findings showed normal tendon tissue. DISCUSSION: No particular motility disturbances or pattern have been found in our patients. Amount of surgery has been chosen according to routine. Amblyopia treatment could be performed as usual. Treatment in our patients with WBS has been similar to ordinary cases of strabismus. Medium-term results do not differ from other squinting patients treated with similar procedures.