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Familial adenomatous polyposis-related desmoid tumors can present with a liquefied center containing gas, accompanied by abdominal pain and sepsis. To date the optimal management of such patients has not been documented.The aim of this study was to review our experience of managing these desmoids grouped together as “intra-abdominal desmoids with air-fluid level” and present a management algorithm.This is a retrospective study of prospectively maintained polyposis registry database.This study was conducted at a tertiary referral center specializing in familial adenomatous polyposis and desmoid disease.Nine patients with intra-abdominal desmoid and air-fluid level were analyzed for the purpose of this study.Two hundred and forty-six patients were identified with desmoid tumor. Of these, a total of 9 patients had an intra-abdominal desmoid with air-fluid level; 7 were women. Age range at diagnosis was 20 to 41 years. The median time from primary surgery to desmoid tumor development was 24 months (range, 0–48 months), and the median time for further progression to air-fluid level was 24 months (range, 0–226 months). Desmoid tumor size ranged from 10 cm to greater than 20 cm in diameter. Two patients were successfully managed with antibiotics alone, and 2 patients were managed with percutaneous drainage and antibiotics. The other 5 patients required surgical intervention involving either excision or drainage with or without proximal defunctioning/exclusion. There was a single 30-day mortality.This study was limited by the small number of patients.The majority of intra-abdominal desmoids with an air-fluid level require surgical intervention. Antibiotics and percutaneous drainage are only successful in a limited number of patients. We present our current treatment algorithm based on this experience.