Acute acalculous cholecystitis (AAC) is a severe disease seen in critically ill patients, including those with autoimmune diseases. We herein report the case of a 41-year-old female who developed macrophage activation syndrome (MAS) accompanied by a recurrence of Kikuchi disease. Abdominal imaging revealed marked thickening of the gallbladder wall and pericholecystic fluid, typically found in AAC. Treatment with intravenous pulse methylprednisolone induced in a significant improvement in the gallbladder wall, resulting in no need for surgical intervention. We should consider that patients with MAS may therefore sometimes develop AAC and that early immunosuppressive therapy can be effective in AAC cases associated with rheumatic or autoimmune diseases.