The diagnosis of pheochromocytoma during pregnancy is uncommon and is at high risk for both mother and baby. We report the case of a 22-year-old woman with MEN2a (mutation C634Y in exon 11 of RET) who had undergone surgery for medullary carcinoma of the thyroid and hyperparathyroidism when she was 18. She was asymptomatic when she was seen at 22 weeks of gestation because of increased urinary metanephrine levels. A 24-h blood pressure monitoring was normal. Abdominal magnetic resonance imaging (MRI) revealed a right-sided, 34x31x28mm, well-limited, adrenal mass with high signal intensity on T2-weighted images; the contralateral adrenal was normal. At 26 weeks of gestation and after an adequate labetalol preparation, a retroperitoneal laparoscopic right-sided adrenalectomy was performed without maternal or foetal complications. Pathohistological examination confirmed the presence of a 3cm pheochromocytoma in the right adrenal gland, with no sign of malignancy. The levels of urinary methoxylated metabolites were normal two months after surgery. The pregnancy progressed normally and the patient delivered a healthy child without complications. In conclusion, firstly, all MEN2a women should be screened for a pheochromocytoma with a 24-h urinary metanephrine and normetanephrine evaluation before or early during pregnancy, even with normal blood pressure; secondly, pheochromocytoma diagnosed during pregnancy should be operated on during pregnancy because of the risks for both mother and baby; thirdly, after medical therapy, retroperitoneal laparoscopic adrenalectomy can be performed during the second trimester of pregnancy.