Background: Autosomal dominant hyper immunoglobulin IgE syndrome is a rare inherited condition that causes immune suppression. Objective: This case report describes a severe case of liver abscess, caused by a cavity-forming infection, secondary to Job’s syndrome. Case presentation: A 25-year-old female patient was emergently referred to the surgical department, for the evaluation of acute, right-sided, upper abdominal pain, fever, and chills. The patient reported a past history of recurrent pulmonary infections as well as a prior diagnosis of Job’s syndrome. An abdominal CT scan revealed a large intrahepatic cystic mass, consistent with a hepatic abscess on the right liver lobe. The patient was started on a course of antibiotics and was admitted to the surgical ward for further treatment. After much deliberation, a multidisciplinary team comprised of general surgeons, gastroenterologists, and interventional radiologists, decided upon the guided drainage of the abscess. Two pigtail catheters were used to drain the cavities. Antibiotic use was de-escalated, the patient gradually recovered, and the reported abscesses were greatly reduced in size. After 14 days of treatment, the patient was successfully released home. Conclusion: In patients with a known history of the autosomal dominant hyper-IgE syndrome, presenting with acute abdominal pain, the liver abscess must be on the top of the differential diagnosis list.