Acute acalculous gallbladder disease is rarely encountered in children. Two observations permit a review of its clinical, diagnostic, and therapeutic aspects. Acute acalculous cholecystitis and acute gallbladder distension present clinically in a similar fashion, although, in the latter the fever is usually absent and there is a history of episodic pain. The pathogenesis of these affections remains uncertain but generalized infection and anomalies of the cystic duct seem to be favoring circumstances. The diagnosis, rarely initially made, could be confirmed by oral cholecystography showing an non visualized gallbladder. Surgery is necessary in order to confirme or refute the diagnosis. The therapeutic approach can be either the simple drainage of the gallbladder or a cholecystectomy. Due to the risk of allowing a cervicocystic obstacle persist, it seems that a cholecystectomy, which is well tolerated by the child, would be preferable.