Acalculous cholecystitis (AC) is a rare disease in children, and its spectrum has not been well established. Twenty-five children with AC were identified (treated between 1970 and 1994) by retrospective clinical and pathological review. The authors recognized two distinct forms of this disease: acute (duration of symptoms <1 month) and chronic (duration >3 months). Thirteen children had acute AC. Seventy-five percent were males; the age range was from 2 months to 20 years. Of these cases, six occurred in the immediate postoperative period, five were in association with a systemic medical illness, and two had an infectious cause ( Salmonella). The mean time of onset of symptoms ranged from 4 to 30 days after surgery or hospitalization (mean, 16 days). All children presented with fever, right-upper-quadrant pain, and vomiting. Other manifestations included jaundice (38%) and right-upper-quadrant mass (23%). Most had leukocytosis (76%) and abnormal liver function test results (62%). Ultrasonography was the most commonly used radiological test, and all 10 cases tested met the ultrasonographic criteria for acute AC. Cholecystectomy was performed in nine children, and pathological examination confirmed cholecystitis. No postoperative complications occurred. The other four children were managed nonoperatively with intravenous antibiotics. One died, but the other three recovered fully. Twelve children had chronic AC. Sixty-seven percent were females; the age range was 7 to 18 years. All presented with chronic symptoms of right-upper-quadrant pain and nausea or vomiting. The leukocyte count and results of liver function tests were normal. Seventy-five percent had evidence of abnormal gallbladder function (noted by a radionuclide hepatobiliary scan or cholecystography). All children in this group underwent cholecystectomy, with pathological confirmation of chronic inflammation. No complications occurred, and all patients had complete resolution of symptoms. The authors conclude that AC in children occurs in two distinct patterns. The acute and chronic forms differ in their clinical setting and presentation. Cholecystectomy is effective treatment of AC, although there may be a role for nonoperative management in selected cases.