Idiopathic perforation of the gallbladder (IPGB) is a rare event, and the underlying mechanisms are unknown. We investigated the clinicopathological characteristics of this disorder. We reported a case of IPGB, and reviewed 30 other cases reported up to the end of 2005 in a Medline and Japan Centra Revuo Medicina search of the medical literature. We analyzed the clinical findings, laboratory data, and histopathological features. The mean age of the patients was 70.2 +/- 12.8 years, and there were 19 men and 12 women. Underlying diseases such as hypertension, cerebral infarction or hemorrhage, renal failure, respiratory failure, and malignancy were reported in 35.5% of the patients. Perforation was found more often in the fundus (53.3%) than in the body (43.3%) or neck (3.3%) of the gallbladder. Thrombus was found in the intramural vessels of the gallbladder wall in 13 patients (50%), whereas 13 (50%) were free of thrombus. Serum amylase was significantly higher in the intramural vessels in the thrombus-negative group than in the thrombus-positive group. The overall operative mortality was 3.3%. Univariate and multivariate analyses indicated that a delay of more than 24 h from the manifestation of symptoms to operation and a perforation size greater than 2 cm required significantly longer hospitalization. Thrombosis in the intramural vessels seems to be related to the events leading to IPGB; however, it is difficult to consider it the only cause. Early operation and the size of the perforation are important determinants of the outcome of treatment for IPGB.