Abstract Background and study aims Cholelithiasis is a rare finding in children, even though recent series show increased detection of this disease. Our objective is to describe epidemiological aspects, clinical aspects and aetiologies as well as to evaluate the management of cholelithiasis in our centre; a paediatric hospital in Sfax in Tunisia. Patients and methods A retrospective study was performed in children with a diagnosis of cholelithiasis between 1979 and 2008 in the paediatric department of Hédi Chaker University Hospital (Sfax, Tunisia). Results Cholelithiasis was detected in 19 patients, including 9 males and 10 females. The median age at diagnosis was 7.8 years (range: 6 months to 15 years). The associated conditions were haemolytic diseases in seven cases; hepatobiliary diseases in five; growth hormone deficiency in two; and dehydration, Down syndrome, immunoglobulin A deficiency and hypocalcaemia in one case. However, in one patient no associated condition was detected. Ultrasound was used for diagnosis in all the patients. Thirteen patients had no cholelithiasis associated symptoms and 6 patients were symptomatizing. The most frequent symptoms were abdominal pain, either with or without vomiting or jaundice. Thirteen patients underwent surgery (open cholecystectomy in seven and laparoscopic cholecystectomy in six patients). In a single patient, immediate surgery was indicated but was not possible because of haemodynamic instability. Conclusion Cholelithiasis is an increasingly recognized disease in children. It is often associated with chronic haemolysis and hereditary hepatobiliary disease in Tunisia. Even though it is more frequently non-symptomatizing, the gallstones must be removed in the majority of cases.