INTRODUCTION: Lower limb varicose vein disease (LLVVD) is the most common vascular disease with a predominant lesion of the main trunks of saphenous veins. At the same time, there exist atypical variants of lesion of the venous system in LLVVD, which cause difficulties in diagnosis and treatment.
 AIM: To study the incidence rate, anatomical bases of the varicose transformation, the features of reflux formation and the results of surgical treatment in atypical forms of LLVVD.
 MATERIALS AND METHODS: The study involved 600 patients with LLVVD, C2-C3 class of clinical manifestations in CEAP classification; 82 of them had atypical forms. The mean age of patients was 40.2 ± 9.2 years, duration of disease 15.0 ± 5.6 years. Duplex scanning of the lower limb venous system was conducted according to Russian recommendations for the diagnosis and treatment of chronic venous disorders of lower limbs of 2018. Patients with atypical forms of LLVVD additionally underwent computed tomography of the venous system with contrast. Surgical treatment of 50 patients with atypical forms of LLVVD included separation of the site of opening of a tributary in the area of saphenofemoral junction after preliminary marking and isolated elimination of varicose tributaries using Muller hooks; incompetent perforating veins were ligated at the epi- or subfascial levels depending on the location. The results were considered in the periods for up to two years.
 RESULTS: According to our data, the incidence of atypical forms of LLVVD was 13.7%. Lesion of the large tributaries of the main saphenous veins accounted for the highest proportion of atypical forms of LLVVD — 68.3%. Of these, varicose transformation of the anterolateral tributary made 98.2%, and of the superficial iliac circumflex vein — 1.8%. Isolated varicose transformation of perforating veins occurred in 31.7% of cases, where transformation of perforating veins of the gluteal area made 7.7%, of perforating veins of the posterolateral surface of the thigh — 46.2%, and of perforating veins of the patella region — 46.2%. The technical success in the postoperative period in the form of elimination of varicose saphenous veins and of the source of their incompetence was achieved in 100% of cases.
 CONCLUSIONS: The incidence of atypical forms of LLVVD is 13.7%, with the main trunks of saphenous veins remaining competent. The anatomical and hemodynamic basis for such forms of LLVVD is incompetence of the deep vein valves, from where the reflux is transmitted to tributaries of the saphenofemoral junction and/or perforating veins of the gluteal region, femoral region or popliteal fossa. Precise separation of varicose tributaries and perforating veins with preservation of the main trunks of subcutaneous veins is an organ-saving method of LLVVD treatment with a good effect in the follow-up period of up to two years.