Objectives. To study bone mineral density (BMD) in cystic fibrosis (CF) children and adults with the CFTR I1234V mutation associated with pancreatic sufficiency. Methods. Lumbar spine, total hip, and whole-body mineral density were measured by dual-energy radiographic absorptiometry (DEXA) scan. Z score was used for those less than 21 years and T score was used for those 21 years or older. Results. Twenty-one CF patients were younger than 21 years and 5 CF patients were 21 years or older. Mean age was 17.29 ± 4.95 years, ranging from 10 to 33 years. The mean BMD Z scores for patients younger than 21 years were −0.69 ± 0.96 (lumbar spine = L1–L4), −0.48 ± 0.92 (total hip), and −0.38 ± 0.86 (total body). The mean T scores for patients 21 years or older were 0.14 ± 0.7 (L1–L4), 0.38 ± 1 (total hip), and 0.52 ± 1.03 (total body). BMD reduction less than −1 was found in 7 (26.9%) CF patients. Vitamin D deficiency in 20 CF patients (76.9%) tended to be lower in CF patients with low BMD. BMD was significantly correlated with FEV1; however, no significant association was observed with P. aeruginosa colonization. Conclusion. BMD reduction does occur in patients with mild CFTR mutation associated with pancreatic sufficiency.