This report describes the case of an 18-month-old Caucasian male infant with clinical and radiological findings indicative of the Schinzel acrocallosal syndrome. He was born to nonconsangiuneous parents. His father had been diagnosed with Greig syndrome. The patient underwent surgery for preaxial polysyndactyly of both hands and feet. The similarity to the Greig syndrome is discussed. It is possible that both the acrocallosal syndrome and the Greig syndrome are variant expressions of the same autosomal dominant condition. Surgery may improve thumb opposition and shoe wear.