A 17-year-old boy presented to our emergency department with an 8-month history of pain and swelling in the left buttock. Physical examination revealed a firm, fixed, large mass, with extension to the thigh with no inflammatory signs.
Magnetic resonance imaging (MRI) showed a mass measuring 17.1 x 14.1 x 10.3 cm located deep within the gluteus maximus muscle and adjacent to the sciatic nerve. The biopsy confirmed the diagnosis of desmoid-type fibromatosis (DTF). He started chemotherapy with metronomic vinorelbine (90 mg per week) with stable disease.

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