Cystic fibrosis (CF) is the most common genetic disorder in Caucasian individuals that primarily manifests asymptomatically in infants. As the disease progresses, CF patients present with multiple organ dysfunctions to include respiratory, gastrointestinal, and reproductive problems. Coloboma is an ocular defect resulting from incomplete or abnormal closure of the primitive embryonic fissure often co-occurs with congenital abnormalities and demonstrates varied genetic expression. The association of CF with coloboma has not been established in current literature. We report the case of a 27-year-old woman with a known history of CF and coloboma and her 21-month-old daughter that presented with both disorders similar to the mother. While CF and colobomas are traditionally regarded as unrelated conditions, this case highlights the potential connection between genetic mechanisms on chromosome 7 and developmental pathways involving the CFTR protein and coloboma-associated genes. Further research is warranted to explore this connection and inform future therapeutic strategies.