Renal angiomyolipoma (AML) is the most common benign tumor of the kidney. There are few case reports in the literature, especially those occurring during pregnancy. We, at this moment, are reporting a case of a 32-year-old female patient who presented at 21 weeks of gestation with right-sided flank pain, chills, macroscopic hematuria, and vomiting. On examination, she was hemodynamically stable, with no fever. Renal ultrasound showed the presence of a hyperechogenic vascularized fatty tissue on the right kidney, measuring 7.4 x 5.1 x 6.2 cms, with minimal pelvicalyceal dilatation. The MRI opted for an angiomyolipoma. Discharged home at day 4 of admission, the patient’s continued the remaining weeks of her pregnancy uneventfully, until 37 weeks. She delivered her baby vaginally with no further complications during pregnancy or in the post-partum period.In conclusion, due to the insufficient data in the literature supporting the management of patients with AML, the individualization of the treatment is an essential strategy.

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