Background: Duane's retraction syndrome is a congenital ocular motility disorder characterized by limitation of abduction and/or adduction of the involved eye. Duane's syndrome is often associated with congenital musculo-skeletal (particularly upper limb) and neural malformations. Case Report: We describe the unusual association of a Type 1 unilateral Duane's with an atraumatic ipsilateral hypoplasia of the deltoid muscle in an otherwise active 14 year old boy. Conclusion: A child with upper limb musculo-skeletal abnormalities should undergo full orthopaedic, medical and ophthalmologic assessment.