This is the case of a male patient who had a road traffic accident at age 9 years. At age 14, he presented with idiopathic limb pain syndrome and lumbar spine pain. He was also having neck and knee pain. Clinical examination at that time revealed pain in the lumbar spine on lightly pressing head (sign) and limited Straight leg rise. Ten fibromyalgia points were tender, and he was generally hypermobile (Beighton’s score 6). Lumboscaral spine x ray showed pars defect. MRI scan was requested and showed congenital absence of inferior vena cava (IVC).  A further MRI lumbar spine was arranged due to persistent back pain. MRI showed other vascular abnormalities. In addition to absent IVC there was increased size of hemiazygos and paravertebral veins and prominent veins at neuro foramina.  This was possibly causing compression of co-existing structures contributing to lower back pain.Case study of a real patient, review of MRI and CT images. For atypical presentation, a literature search was done.Case study of a practice patient.Congenital absence of inferior vena cava usually presents with deep vein thrombosis. The patient was treated with high dose of painkillers for back pain. In this case there was delay in correlating back pain with congenital absence of inferior vena cava.Congenital anomaly like total absence or atresia of inferior vena cava is a rare condition. The incidence of absence inferior vena cava is 0.2-0.6% in healthy individual. As the collateral circulation through azygous and portal vein maintains sufficient venous return so patients are often asymptomatic. The condition may predispose to venous stasis leading to varicose veins and deep vein thrombosis.In this case the patient developed lower limb pain and back pain from an early age. He was found not to have DVT and his thrombophilia screen was negative. It is possible that his symptom of leg pain was due to increased demand  on venous system and stasis and the back pain due to increased endoneurial pressure.