Angiomyomatous hamartoma (AMH) of the lymph node is an extremely rare, benign vascular disease of unknown etiology. It is characterized by partial or complete replacement of the lymph node parenchyma by irregularly distributed, thick-walled blood vessels, smooth muscle bundles and adipose tissue in a fibrotic stroma. Angiomyomatous hamartoma occurs mainly in inguinal and femoral nodal regions, but there are a few reports of some other locations – submandibular, cervical, popliteal and paraaortic lymph nodes. We present a case of a 37-old female patient with AMH in the pelvic and paraaortic lymph nodes who presented with weight loss – 7 kg in 7 months. The differential diagnosis of AMH includes lymphangiomyomatosis, which, unlike AMH, involves primarily thoracic and intra-abdominal lymph nodes: nodal leiomyomatosis with less pronounced vascular proliferation and angiomyolipoma of the lymph node. The latter is composed of the same tissues as in AMH, but the smooth muscle component shows increased cellularity, polymorphism and increased mitotic activity, as well as a typical immune profile with coexpression of melanocyte markers and estrogen, which were negative in our case. The world literature references show that this is the first reported case in which the disease manifested itself with weight loss and affected paraaortic lymph nodes in a female patient.