Fibrolipomatous Hamartoma of the Median Nerve with Macrodystrophia Lipomatosa

Fibrolipomatous hamartoma (FLH) is a rare congenital condition that presents with a benign overgrowth of the bone and fibroadipose tissue termed as macrodystrophia lipomatosa (MDL). Although commonly seen in the median nerve, other peripheral nerves can be involved. Diagnosis can be made on magnetic resonance imaging (MRI) due to the characteristic coaxial cable appearance on axial images and the spaghetti appearance on sagittal images. Histology shows mature adipose and fibrous tissue infiltrating the epineural and perineural compartments. Multiple or debulking surgeries are often needed, with an emphasis on cosmetic aspects. We present one such case in which wide margin excision and sural nerve graft were carried out.