Cystic fibrosis (CF) is a life-shortening genetic disease with many treatment requirements that necessitate the participation of a caregiver, especially if the patient is a child. We performed an integrated literature review of original research evaluating quality of life in caregivers of patients with CF. We searched the terms quality of life, cystic fibrosis, and caregivers in the MEDLINE, CINAHL, Child Development and Adolescent Studies, PsycARTICLES, and Psychology and Behavioral Sciences Collection databases from 2007 to 2017. Nine articles were included and showed that caregiver quality of life is affected by caring for a child with CF as evidenced by high rates of anxiety and depression. Anxiety and depression in caregivers affects adherence to the child's treatment regimen, causing detrimental effects on the health of the child. Screening for depression and anxiety and referral for treatment when necessary are needed to optimize family health. Findings include 1) CF has numerous treatment burdens for caregivers, 2) rates of anxiety and depression are two to three times higher in caregivers of patients with CF than in the general population, 3) depressed parents are less likely to adhere to their child's treatment regimen, and 4) few CF families report having received mental health screening or treatment.