Introduction: Lipoblastoma is a benign neoplasia of the adipose tissue. It is a rare conditionand almost exclusively presents in children under 3 years old. It usually occurs in extremities as a painless volume increase of progressive growth, with the definitive diagnosis being established by pathological and cytogenetic analysis. The treatment of choice is complete resection, and follow-up period of up to five years is recommended due to a recurrence of up to 25%., Objective: To present an unusual location of this uncommon condition in an infant, and review the related literature., Case Report: A sixteen-month child with an increase in abdominal growth of six-months progression, associated with a decreased food intake, and with no other symptoms. The imaging study revealed a lipoid-like image compromising almost the entire abdominal cavity, very suggestive of lipoblastoma. A resection was performed on an 18cm diameter retroperitoneal tumour that rejected the adjacent organs. Histological analysis was enough to confirm diagnosis without the need for cytogenetic analysis. The follow-up showed no recurrence of the disease., Conclusion: Given the rarity of this disease and its unusual presentation, we communicate this clinical case, in order to be considered in the differential diagnosis of abdominal mass in chilhood., (Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.)