•Extrapulmonary lymphangioleiomyomatosis is rare and can be associated with tuberous sclerosis.•Recognition of lymphangioleiomyomatosis is important for early disease screening and genetic testing.•Lymphangioleiomyomatosis in lower uterine segment is very rare and can be overlooked.
Keywords: Lymphangioleiomyomatosis; Tuberous sclerosis; Uterus.
© 2021 The Author(s).