Lipofibromatosis is a relatively rare pediatric neoplasm, which usually manifests as an ill-defined soft tissue mass involving the upper and lower distal extremities, the trunk, and, less frequently, the head. To date, no cytogenetic abnormalities have been reported in this uncommon neoplasm. We present a case of lipofibromatosis featuring a three-way t(4;9;6) translocation in a 5-year-old boy.