Background: Torsion of kidney transplant refers to rotation of the kidney transplant graft around its vascular pedicle resulting in vascular compromise and infarction. It is a rare complication of kidney transplantation associated with a high rate of graft loss. Clinical presentation and diagnostic imaging modalities are non-specific, and surgical exploration is therefore often delayed. Methods: We present a case report and review of the literature. Studies were identified by searching Medline and Embase from January 1954 to December 2010. Data was extracted regarding the clinical presentation, investigation, findings on surgical exploration, and treatment outcomes of patients with torsion of kidney transplant. Results: Eight manuscripts with 16 cases of kidney torsion were found. Presenting symptoms were decreased renal function (13 cases), abdominal pain (10 cases), oliguria/anuria (9 cases), nausea and vomiting (4 cases), fever (3 cases), diarrhoea (3 cases), weight gain (2 cases), oedema (3 cases), fatigue (1 case) and impalpable graft (1 case). Investigations were Doppler sonography (11 cases), grey-scale sonography (7 cases), nuclear scintigraphy (5 cases), computed tomography scan (4 cases), and magnetic resonance imaging/magnetic resonance angiography (1 case). Of the 16 published cases of torsion, seven (44%) grafts were detorted and salvaged, three (19%) grafts were detorted but subsequently lost and six (38%) patients underwent immediate nephrectomy. Conclusions: A prompt consideration of the diagnosis of torsion of kidney transplant is required to prevent delay in surgical intervention. We recommend urgent Doppler ultrasound be used as first-line investigation, followed by prompt surgical exploration. We recommend the use of prophylactic nephropexy to prevent torsion.