© 2020 Associação Brasileira de Pesquisa e Pós-Graduação em FisioterapiaBackground: Pulmonary and upper limbs function of children with Duchenne muscular dystrophy (DMD) are known to deteriorate throughout the disease process. However, there is a lack of information on the extent of impairments in the early stages of DMD when compared to healthy peers. Objective: To investigate to what extent pulmonary and upper limbs function of children with early stage DMD are impaired. Methods: Sixty-one children participated in the study: 31 with Grade 1 DMD (study group) according to the Brooke Upper and Lower Extremity Functional Classification Systems, and 30 age matched healthy peers (control group). Pulmonary function was determined with pulmonary function tests. The Performance of Upper Limb test was used to evaluate the upper limbs function. Results: Study and control groups were homogenous in terms of physical characteristics (p > 0.05). Pulmonary and upper limbs function of children with DMD were about 85% and 93% of healthy peers, respectively. Conclusion: This study provides evidence for deterioration of pulmonary and upper limbs function in children with early stage DMD. Better knowledge of deterioration rate over time may help therapists to better plan and update their plan of care.