Introduction. Autosomal recessive polycystic kidney disease (ARPKD) is a rare genetic disorder that affects the kidneys and, in some cases, the liver. CT with contrast (CECT) has been found to be an effective method to identify complications associated with ARPKD. Material and Methods. Retrospective analysis of patients treated for ARPKD at the Institute of Mother and Child in Republic of Moldova between 2018 and 2023. The study included 6 patients, of which 1 in 2018, 2 in 2019, 1 in 2021, and 2 in 2023. Results. All patients underwent CECT, were was detected renal and hepatic conditions. Regarding the patients diagnosed with ARPKD at the institution, all 6 patients (100%) were female, of which 3 patients (50%) were between 0-7 years old and 3 patients (50%) were between 8-13 years old. CECT identified that 5 patients (83.33%) had renal cysts, 5 patients (83.3%) had portal hypertension, 5 patients (83.33%) had Caroli syndrome, 5 patients (83.3%) had splenomegaly, and 3 patients (50%) had hepatic fibrosis. Conclusions. Understanding ARPKD pathology is essential for doctors, researchers, and patients because this condition can equally affect the kidneys and liver, being a rare and potentially devastating disease. In the last 5 years, only female children up to 13 years old have been referred to the Institute of Mother and Child in Republic of Moldova, with an incidence of 1-2 patients per year. The use of CECT has proven to be useful in the evaluation and management of patients with this condition, thus obtaining early diagnosis and treatment, preventing serious complications and improving the prognosis of patients.