The desmoplastic small round cell tumour is a rare neoplasia, with diagnostic complexity and a lethal course. Its clinical presentation is unspecific. Histologically, it is classified as an aggressive soft tissue sarcoma that shares similar characteristics with the family of the small and blue cells tumours.

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Lipofibromatous hamartoma is an uncommon tumor that causes nerve enlargement due to fatty adipose tissue infiltration in peripheral nerves being the median nerve at the carpal tunnel the most commonly affected site, typically associated with macrodactyly. Here we present a case which affected a digital branch of the median nerve