Dysphagia in a child with aggressive fibromatosis of the esophagus

A case of a girl who developed severe dysphagia from an aggressive fibromatosis involving the carvical esophagus is reported. A 3×4 cm unencapsulated fibrous tumor was completely excised with the involved esophageal wall. The esophageal defect was bridged by an inverted skin tube with good functional and cosmetic results. Eight years later the patient is disease-free. Primary radical excision is the treatment of choice of aggressive fibromatosis.