Lipofibromatosis.

Lipofibromatosis is a benign pediatric soft tissue tumor arising preferentially in the distal extremities. Histologically, the tumor shows abundant adipose tissue admixed with a spindle cell component, often concentrated in septal and perimysial locations. The index case is being presented to discuss the histopathological and immunohistochemical clues to differentiate it from other fibrofatty tumors of childhood. An 11-month-old male child presented with a slowly growing mass on the upper back. MRI findings were suggestive of an adipocytic tumor. Microscopy revealed a lesion composed of mature adipocytes and intervening fibrous bands with infiltration into the adjacent skeletal muscle, features of lipofibromatosis. Lipofibromatosis should be considered in the differential diagnosis of a pediatric fibrofatty tumor. Accurate diagnosis is essential for proper patient management as incomplete removal of the tumor may result in recurrence.