Background: Servelle-Martorell syndrome is a rare venous malformation rarely involving arterial system and is associated with skeletal abnormalities. Case Report: A 25 year young male presented with progressive enlargement of left upper limb and shoulder girdle since birth. Multiple grotesque, soft, swollen areas were observed over the whole of the arm upto the shoulder girdle. X ray and ultrasound were suggestive of soft tissue swellings with phleboliths in the affected upper limb and periscapular region with hypoplastic distal bones. Color Doppler showed mildly reduced arterial blood flow with normal spectral analysis. A contrast enhanced CT study showed multiple superficial dilated veins calcified intraluminal areas suggestive of phelboliths, hypotrophic muscles, thinning of radius and ulna and normal arterial system. Conclusion: Early diagnosis of the Servelle - Martorell syndrome can help in reducing the morbidity of the patient along with associated complications.