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Cilj: Sindrom Klippel-Trenaunay-Weber rijedak je kongenitalni sporadični poremećaj koji rezultira abnormalnim razvojem krvnih žila, mekih tkiva i kostiju. Cilj je ovog rada prikazati izazovan tijek dijagnostike i terapije u pacijentice s ovom rijetkom bolešću, gdje je izostao kožni hemangiom, inače prisutan u 98 % pacijenata i gdje se bolest javila na gornjem ekstremitetu, što je zabilježeno u tek 12,5 % slučajeva. Prikaz slučaja: U ženskog je novorođenčeta pri prvom pregledu neonatologa uočena bezbolna oteklina desne ruke i kroz kožu vidljive zadebljane vene na desnoj podlaktici i šaci. Promjene su ostale trajno prisutne uz blago ograničenu fleksiju lakatnog zgloba i prstiju desne ruke. Nakon sedam godina javili su se bolovi u desnoj ruci pa je učinjena ehotomografija mišića podlaktice i šake. Nalazi su pokazali široke venske prostore uz znakove arteriovenskih fistula, nakon čega je upućena na CT s kontrastom i flebografiju. Potvrđena je tvorba vaskularnog podrijetla, dijelom trombozirana i niske brzine protoka. U idućih šest godina tegobe su postale jače izražene, stoga je bio indiciran kirurški tretman stanja. Pacijentica je podvrgnuta ekstirpaciji varikoziteta desne ruke. Tegobe su i dalje perzistirale, stoga su učinjene magnetska rezonancija i angiografija magnetskom rezonancijom desne ruke, čiji su nalazi bili suspektni na intramuskularni hemangiom. Sedam godina kasnije učinjena je posljednja embolizacija vaskularne tvorbe te se planira nastavak operativnog liječenja. Zaključak: Sindrom Klippel-Trenaunay može se prezentirati bez kožnog hemangioma, koji je inače najčešći klinički znak. Također, znakovi sindroma mogu atipično biti prisutni na gornjim ekstremitetima., Aim: Klippel-Trenaunay-Weber syndrome is a rare congenital sporadic disorder which results in abnormal development of blood vessels, soft tissue and bones. The aim of this case report is to present a challenging course of diagnostics and treatment in patients with this rare disease, where skin hemangioma, otherwise present in 98%, was absent and disease occurred in the upper extremity, which was recorded in only 12,5% of cases. Case report: The first examination of a neonatologist in a female newborn showed painless swelling of the right arm and visible varicose veins on the right forearm and hand. The changes were permanent and with mild flexion restriction of elbow joint and right hand fingers. After seven years the pain appeared in the right arm and the echotomography of the forearm and hand muscles was done. The results showed broad veins and signs of arteriouvenous fistulas, after which the computed tomography with contrast and flebography were ordered. The presence of a structure of vascular etiology was confirmed, partially thrombosed, with slow flow. In the next six years simptoms advanced and surgical treatment was recommended. The patient has undergone varicosities extirpation. The pain persisted, therefore magnetic resonance and magnetic resonance angiography of the right arm were indicated and suspected of intramuscular hemangioma. Embolisation of vascular malformation was performed seven years later and there are plans for further surgical treatment. Conclusion: Klippel-Trenaunay syndrome may occur without cutaneous hemangioma, which is otherwise the most common clinical sign. Also, syndrome signs could atypically be found in the upper extremities.