Lymphangioleiomyomatosis (LAM) is a rare condition usually occurring in young women of childbearing age. It is characterised by the presence of abnormal smooth muscle cells (LAM cells) in the lungs, lymph nodes, and/or other organs, with a few reports of isolated extrapulmonary cases. We report the case of a 26-yr-old female who presented with a painless shoulder mass. Ultrasonography, computed tomography and magnetic resonance imaging revealed an intermuscular mass with cystic and solid tissue components, measuring 6 cm x 4 cm. The mass was removed surgically after the diagnosis of LAM was made following tru-cut biopsy. This case corresponds to an exceptional localisation of LAM.

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