Intracranial desmoid tumors are extremely rare. The association of desmoid tumors with familial adenomatous polyposis coli was reported previously, with the tumors involving trunk and extremities. We report a 3.5-year-old girl with intracranial desmoid tumor with familial adenomatous polyposis coli. This condition in a child is rarely reported. Followup of the patient after cranial surgery and of the family for this premalignant inherited condition is necessary. Copyright (c) 2008 S. Karger AG, Basel.