Servelle-Martorell syndrome (SMS) is a rare congenital anomaly that is frequently mistaken for Klippel-Trenaunay syndrome (KTS) or Parkes-Weber syndrome (PWS). SMS usually involves venous dilatations, soft tissue hypertrophy, and bone hypotrophy, while KTS and PWS usually have bone hypertrophy. The management of SMS is primarily conservative, and surgery should be done selectively. This study aimed to report a case of SMS and its management to relieve a painful aneurysm on the right knee by excision.

Methods: A 16-year-old male patient presented with a slightly shorter right lower limb and multiple bluish swelling on his right lower limb. Supporting venography and angiography showed venous malformations, soft tissue hypertrophy, and bone hypotrophy on the right lower limb. The physical and supporting examinations led to the diagnosis of SMS. The patient was admitted because of severe pain in the right knee. Surgical excision of the venous malformation in the knee region was done to relieve the pain. The patient felt significantly reduced pain on one-month follow-up. SMS has similar features to KTS and PWS. The excision surgery was indicated due to severe pain in the right knee.

Conclusions: SMS is a rare disease and important to be recognized as it is frequently mistaken as KTS or PWS. The management is primarily conservative and surgical management should only be done in severe aneurysmal complications and shunting. As venous malformations and pain can reoccur after surgical excision, regular follow-ups should be maintained.