Objective To investigate the characteristics and causes of misdiagnosis of periorbital lymphatic malforma-tion.Methods The clinical data of a child with periorbital lymphatic malformation combined with venous malformation who was misdiagnosed as arteriovenous malformation and treated in 2023 were retrospectively analyzed.Results Swelling of the right eye was found in this child after birth,which was misdiagnosed as hemangioma and arteriovenous malformation succes-sively.Sclerotic treatment and glue embolization of arteriovenous malformation tissue were performed respectively,and the symptoms were relieved to some extent,which were intermittent and recurrent.The patient was diagnosed with lymphatic mal-formation combined with venous malformation after performing magnetic resonance imaging examination and extraction of lym-phatic fluid during ultrasound-guided local puncture of the periorbital capsule.The misdiagnosis lasted 11 years.After diagno-sis and treatment with sclerotherapy combined with Sirolimus treatment,the patient's symptoms were significantly relieved and no recurrence occurred during follow-up.Conclusion Periorbital lymphatic malformation combined with venous malformation is more likely to be misdiagnosed,and joint imaging examination and multidisciplinary team evaluation are needed to reduce misdiagnosis.