Cystic fibrosis (CF) is the most common life-limiting autosomal recessive disorder. While gastrointestinal and pulmonary complications are frequent in CF, the coexistence of malignancy is rare. We aimed to present a case of bilateral Wilms’ tumor (BWT) in a child with CF and to review the relevant literature. A female child diagnosed with CF in the neonatal period was referred to our clinic at the age of 2 years, when bilateral Wilms’ tumor was detected. Clinical history, laboratory findings, and imaging results were evaluated, and the diagnostic, therapeutic, and follow-up process were described in detail. In addition, a literature review was performed to identify similar cases.In addition to the typical clinical manifestations of CF, abdominal imaging revealed bilateral Wilms’ tumor. The clinical course and treatment process of the patient were reported. Literature review demonstrated that the coexistence of CF and Wilms’ tumor is extremely rare, with only a limited number of cases described. Although malignancy is rare in children with CF, it should not be overlooked. This case highlights the unusual coexistence of CF and Wilms’ tumor and emphasizes that reporting such cases provides a valuable contribution to the literature. Clinicians should consider the possibility of malignancy during the long-term follow-up of patients with CF, particularly as survival improves.