This paper describes a case of liposarcoma of exceptional paratesticular location (first in the literature described in infancy). The case corresponds to a 5-year-old boy. The influence of the histology, location of the tumour and possibility of complete surgical exeresis is fundamental with regard to prognosis and evolution. Pediatric experience is extremely limited and we may superimpose the treatment in children of that carried out in adults (more case histories), in whom multimodal therapy (surgery, radiotherapy, chemotherapy) concludes with encouraging results. The updating of previously published cases reveals the predominance of myxoid histology, a lower index of relapses in infancy and a high peak of incidence in early infancy.
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