We present the morphometric characteristics of the diaphragm in a patient with Ullrich congenital muscular dystrophy. A 11-year-old girl was referred to our hospital for regular assessment of respiratory function. She has experienced severe dyspnea when being infected with upper respiratory tract infection since the age of 9 years. Ultrasonography could reveal patchy areas of increased echogenicity in the diaphragm bright and speckled echo pattern and a reduced diaphragm thickness. Sonographic parameters of diaphragm add information in the assessment and follow up of children with respiratory dysfunction due to muscle diseases.