Abnormal body composition in myotonic dystrophy type 1 (DM1) are affected by energy intake above resting energy expenditure (REE). We aim to investigate the characteristics and relationship between body composition, REE, and pulmonary function in patients with DM1, and to examine their changes in 1 year. The study design was a single-center, cross-sectional, and longitudinal study of body composition, REE characteristics, and pulmonary function. Twenty-one male patients with DM1 and 16 healthy volunteers were registered in the study. Body composition was measured using dual-energy X-ray absorptiometry (DEXA). Fat mass (FM) index (kg/m 2 ), fat-FM index (kg/m 2 ), and skeletal mass index (kg/m 2 ) were calculated. The measurements were taken breath by breath with a portable indirect calorimeter. The REE was calculated using the oxygen intake (VO 2 ) and carbon dioxide output (VCO 2 ) in the Weir equation. Basal energy expenditure (BEE) was calculated by substituting height, weight, and age into the Harris-Benedict equation. The study enrolled male patients with DM1 (n = 12) and healthy male volunteers (n = 16). Patients with DM1 (n = 7) and healthy volunteers (n = 14) could be followed in 1 year. The body composition of patients with DM1 was significantly higher in the FM index and significantly lower in the fat-FM index and skeletal mass index. The REE of patients with DM1 was significantly lower and was not associated with body composition. Patients with DM1 had poor metabolism that was not related to body composition. FM was high and lean body mass was low. Abbreviations: BEE= basal energy expenditure, BMI = body mass index, BW = body weight, CONUT = controlling nutritional status, CPF = cough peak flow, CTG = cytosine-thymine-guanine, DEXA = dual-energy X-ray absorptiometry, DM1 = myotonic dystrophy type 1, FEV1 = forced expiratory volume in 1 second, FFMI = fat free mass index, FM = fat mass, FMI = fat mass index, FVC = forced vital capacity, HR = heart rate, LBM = lean body mass, MIRS = muscular disability rating scale, REE = resting energy expenditure, RR = respiratory rate, SMI = skeletal mass index, TV = tidal volume, VC = vital capacity, VCO 2 = carbon dioxide output, VE = minute ventilation, VO 2 /HR = O 2 pulse, VO 2 = oxygen intake.