Congenital lipofibromatosis is a rare slow growing benign fibrofatty neoplasm presenting in childhood. The case of a boy presenting soon after birth with diffuse lower extremity enlargement associated with a significant tibial deformity is presented. Magnetic resonance imaging and soft tissue biopsy confirmed the diagnosis of lipofibromatosis. The child started having problems with his gait after the age of two. The tibial bowing was corrected gradually using a Taylor Spatial Frame resulting in a good clinical outcome.
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