Introduction:

Pulmonary lymphangioleiomyomatosis constitutes a challenge, both in its diagnostic management and in the care of complications. It is a rare disease characterized by the proliferation of smooth muscle around the bronchovascular and lymphatic structures and in the pulmonary interstitium, together with cystic dilation of the terminal air spaces.

Objective:

To describe a diagnosed case of pulmonary lymphangioleiomyomatosis.

Case report:

A case of pulmonary lymphangioleiomyomatosis is reported, which was clinically showed as recurrent pneumothorax. A definitive diagnosis was reached by performing a lung biopsy.

Discussion:

Lymphangioleiomyomatosis is a rare entity that mainly affects women of childbearing age and is characterized by the presence of a diffuse cystic pattern. Its prevalence and incidence is unknown.

Conclusions:

It can be concluded that the presence of spontaneous or recurrent pneumothorax in a woman of childbearing age, or pregnant, should be suspected lymphangioleiomyomatosis.

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