The authors report a rare case of acute emphysematous cholecystitis with pneumoperitoneum. Emphysematous cholecystitis is an uncommon variant of acute cholecystitis. Association with pneumoperitoneum is very rare and the finding of a macroscopic perforation of the gallbladder is possible only in a few cases. A review of the literature revealed 15 other cases of this combination. Diagnostic options and treatment modalities in these patients are discussed here.