Congenital muscular torticollis (CMT) is a disorder characterized by a shortening of at least one of the cervical muscles and tilting of the head to the opposite side. The most commonly affected muscle is the sternocleidomastoid (SCM) muscle. An unusual case is presented here: a CMT patient with involvement of the trapezius muscle in addition to the SCM muscle who has recently been treated at North Carolina Memorial Hospital. This patient still has a slight residual deformity despite two surgical procedures, physical therapy, and splinting. A review of 41 CMT patients who presented to this institution from 1952 to the present demonstrates that the disorder is extremely variable in terms of severity of deformity and response to various types of therapy. The condition will resolve spontaneously in the majority of patients by the age of 1 year, and if the condition has not resolved by then, it is unlikely to do so without surgery. We therefore believe that surgery should be postponed until after 1 year of age. Most of the patients requiring surgery are effectively treated by division of the involved muscle in at least one area with or without lengthening of the SCM muscle. Splinting and physical therapy are recommended postoperatively. A few patients such as the unusual one with involvement of the trapezius muscle resist our most aggressive therapeutic efforts.